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1. Does the impact of COVID-19 on patients with systemic sclerosis change over time?

Author: MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Deibel, Elisabeth, Carreira, Patricia E, Vonk, Madelon, Del Papa, Nicoletta, Bečvář, Radim, Guillén-Del-Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria-Grazia, Giollo, Alessandro, Mekinian, Arsène, de Vries-Bouwstra, Jeska, De Santis, Maria, Balbir-Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose-Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre-Sancho, Juan J, Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez-Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie-Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, Del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E, Matucci, Marco, Gabrielli, Armando, Distler, Oliver, Hoffmann-Vold, Anna-Maria, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Deibel, Elisabeth, Carreira, Patricia E, Vonk, Madelon, Del Papa, Nicoletta, Bečvář, Radim, Guillén-Del-Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria-Grazia, Giollo, Alessandro, Mekinian, Arsène, de Vries-Bouwstra, Jeska, De Santis, Maria, Balbir-Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose-Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre-Sancho, Juan J, Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez-Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie-Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, Del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E, Matucci, Marco, Gabrielli, Armando, Distler, Oliver, and Hoffmann-Vold, Anna-Maria
Published: 2024

2. Patient Assessment Chronic Illness Care (PACIC) and its associations with quality of life among Swiss patients with systemic sclerosis: a mixed methods study

Author: Kocher, Agnes; https://orcid.org/0000-0002-8716-9727, Simon, Michael, Dwyer, Andrew A, Blatter, Catherine, Bogdanovic, Jasmina, Künzler-Heule, Patrizia, Villiger, Peter M, Dan, Diana, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Nicca, Dunja; https://orcid.org/0000-0002-4133-8177, Kocher, Agnes; https://orcid.org/0000-0002-8716-9727, Simon, Michael, Dwyer, Andrew A, Blatter, Catherine, Bogdanovic, Jasmina, Künzler-Heule, Patrizia, Villiger, Peter M, Dan, Diana, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, and Nicca, Dunja; https://orcid.org/0000-0002-4133-8177
Abstract: BACKGROUND: The Chronic Care Model (CCM) is a longstanding and widely adopted model guiding chronic illness management. Little is known about how CCM elements are implemented in rare disease care or how patients' care experiences relate to health-related quality of life (HRQoL). We engaged patients living with systemic sclerosis (SSc) to assess current care according to the CCM from the patient perspective and their HRQoL. METHODS: We employed an explanatory sequential mixed methods design. First, we conducted a cross-sectional quantitative survey (n = 101) using the Patient Assessment of Chronic Illness Care (PACIC) and Systemic Sclerosis Quality of Life (SScQoL) questionnaires. Next, we used data from individual patient interviews (n = 4) and one patient focus group (n = 4) to further explore care experiences of people living with SSc with a focus on the PACIC dimensions. RESULTS: The mean overall PACIC score was 3.0/5.0 (95% CI 2.8-3.2, n = 100), indicating care was 'never' to 'generally not' aligned with the CCM. Lowest PACIC subscale scores related to 'goal setting/tailoring' (mean = 2.5, 95% CI 2.2-2.7) and 'problem solving/contextual counselling' (mean = 2.9, 95% CI 2.7-3.2). No significant correlations were identified between the mean PACIC and SScQoL scores. Interviews revealed patients frequently encounter major shortcomings in care including 'experiencing organized care with limited participation', 'not knowing which strategies are effective or harmful' and 'feeling left alone with disease and psychosocial consequences'. Patients often responded to challenges by 'dealing with the illness in tailored measure', 'taking over complex coordination of care' and 'relying on an accessible and trustworthy team'. CONCLUSIONS: The low PACIC mean overall score is comparable to findings in patients with common chronic diseases. Key elements of the CCM have yet to be systematically implemented in Swiss SSc management. Identified gaps in care related to lack of shared d
Published: 2023

3. Patient preferences for the treatment of systemic sclerosis-associated interstitial lung disease: a discrete choice experiment

Author: Bruni, Cosimo, Heidenreich, Sebastian, Duenas, Ashley, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Allanore, Yannick, Chatelus, Emmanuel, Distler, Joerg H. W., Hachulla, Eric, Hsu, Vivien M., Hunzelmann, Nicolas, Khanna, Dinesh, Truchetet, Marie-Elise, Walker, Ulrich A., Alves, Margarida, Schoof, Nils, Saketkoo, Lesley Ann, Distler, Oliver, Bruni, Cosimo, Heidenreich, Sebastian, Duenas, Ashley, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Allanore, Yannick, Chatelus, Emmanuel, Distler, Joerg H. W., Hachulla, Eric, Hsu, Vivien M., Hunzelmann, Nicolas, Khanna, Dinesh, Truchetet, Marie-Elise, Walker, Ulrich A., Alves, Margarida, Schoof, Nils, Saketkoo, Lesley Ann, and Distler, Oliver
Abstract: Objectives Treatments for SSc-associated interstitial lung disease (SSc-ILD) differ in attributes, i.e. mode of administration, adverse events (AEs) and efficacy. As physicians and patients may perceive treatments differently, shared decision-making can be essential for optimal treatment provision. We therefore aimed to quantify patient preferences for different treatment attributes. Methods Seven SSc-ILD attributes were identified from mixed-methods research and clinician input: mode of administration, shortness of breath, skin tightness, cough, tiredness, risk of gastrointestinal AEs (GI-AEs) and risk of serious and non-serious infections. Patients with SSc-ILD completed an online discrete choice experiment (DCE) in which they were asked to repeatedly choose between two alternatives characterized by varying severity levels of the included attributes. The data were analysed using a multinomial logit model; relative attribute importance and maximum acceptable risk measures were calculated. Results Overall, 231 patients with SSc-ILD completed the DCE. Patients preferred twice-daily oral treatments and 6-12 monthly infusions. Patients' choices were mostly influenced by the risk of GI-AEs or infections. Improvement was more important in respiratory symptoms than in skin tightness. Concerning trade-offs, patients accepted different levels of increase in GI-AE risk: +21% if it reduced the infusions' frequency; +15% if changing to an oral treatment; up to +37% if it improved breathlessness; and up to +36% if it reduced the risk of infections. Conclusions This is the first study to quantitatively elicit patients' preferences for treatment attributes in SSc-ILD. Patients showed willingness to make trade-offs, providing a firm basis for shared decision-making in clinical practice.
Published: 2022

4. Representativeness of Systemic Sclerosis Patients in Interventional Randomized Trials: an analysis of the EUSTAR database

Author: Iudici, Michele, Jarlborg, Matthias, Lauper, Kim; https://orcid.org/0000-0002-4315-9009, Müller-Ladner, Ulf, Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Balbir-Gurman, Alexandra, Doria, Andrea, Airò, Paolo, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Riccieri, Valeria, Vonk, Madelon C; https://orcid.org/0000-0002-2266-9907, Gabrielli, Armando, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Szücs, Gabriella, Martin, Thierry, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Courvoisier, Delphine S, EUSTAR collaborators, Iudici, Michele, Jarlborg, Matthias, Lauper, Kim; https://orcid.org/0000-0002-4315-9009, Müller-Ladner, Ulf, Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Balbir-Gurman, Alexandra, Doria, Andrea, Airò, Paolo, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Riccieri, Valeria, Vonk, Madelon C; https://orcid.org/0000-0002-2266-9907, Gabrielli, Armando, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Szücs, Gabriella, Martin, Thierry, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Courvoisier, Delphine S, and EUSTAR collaborators
Abstract: OBJECTIVE: To estimate the extent of and the reasons for ineligibility in randomized controlled trials (RCTs) of systemic sclerosis (SSc) patients included in the EUSTAR database, and to determine the association between patient's features and generalizability of study results. METHODS: We searched Clinicaltrials.gov for all records on interventional SSc-RCTs registered from January 2013 to January 2018. Two reviewers selected studies, and information on the main trial features were retrieved. Data from 8046 patients having a visit in the EUSTAR database since 2013 were used to check patient's eligibility. The proportion of potentially eligible patients per trial, and the risk factors for ineligibility were analyzed. Complete-, worst- and best-case analyses were performed. RESULTS: Of the 37 RCTs included, 43% were conducted in Europe, 35% were industry-funded, and 87% investigated pharmacological treatments. Ninety-one percent of 8046 patients included could have participated in at least one RCT. In complete-case analysis, the median [range] proportion of eligible patients having the main organ complication targeted by each study was 60% [10-100] in the overall sample of trials, ranging from 50% [32-79] for trials on skin fibrosis to 90% [34-77] for those targeting Raynaud's phenomenon. Among the criteria checked, treatment- and safety-related but not demographic were the main barriers to patient's recruitment. Older age, absence of Raynaud's phenomenon, and lower mRSS were independently associated with the failure to fulfill criteria for any of the included studies. CONCLUSIONS: Patient's representativeness in SSc-RCTs is highly variable and is driven more by treatment- and safety-related rather than demographic criteria.
Published: 2022

5. Patient preferences for the treatment of systemic sclerosis-associated interstitial lung disease: a discrete choice experiment

Author: Bruni, Cosimo; https://orcid.org/0000-0003-2813-2083, Heidenreich, Sebastian; https://orcid.org/0000-0002-1909-5770, Duenas, Ashley, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Gabrielli, Armando, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Chatelus, Emmanuel, Distler, Jörg H W; https://orcid.org/0000-0001-7408-9333, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Hsu, Vivien M, Hunzelmann, Nicolas, Khanna, Dinesh; https://orcid.org/0000-0003-1412-4453, Truchetet, Marie-Elise, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Alves, Margarida, Schoof, Nils, Saketkoo, Lesley Ann, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Bruni, Cosimo; https://orcid.org/0000-0003-2813-2083, Heidenreich, Sebastian; https://orcid.org/0000-0002-1909-5770, Duenas, Ashley, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Gabrielli, Armando, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Chatelus, Emmanuel, Distler, Jörg H W; https://orcid.org/0000-0001-7408-9333, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Hsu, Vivien M, Hunzelmann, Nicolas, Khanna, Dinesh; https://orcid.org/0000-0003-1412-4453, Truchetet, Marie-Elise, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Alves, Margarida, Schoof, Nils, Saketkoo, Lesley Ann, and Distler, Oliver; https://orcid.org/0000-0002-0546-8310
Abstract: OBJECTIVES: Treatments for systemic sclerosis-associated interstitial lung disease (SSc-ILD) differ in attributes, i.e. mode of administration, adverse events (AEs) and efficacy. As physicians and patients may perceive treatments differently, shared decision-making can be essential for optimal treatment provision. We therefore aimed to quantify patient preferences for different treatment attributes. METHODS: Seven SSc-ILD attributes were identified from mixed-methods research and clinician input: mode of administration, shortness of breath, skin tightness, cough, tiredness, risk of gastrointestinal AEs (GI-AEs), and risk of serious and non-serious infections. Patients with SSc-ILD completed an online discrete choice experiment (DCE) in which they were asked to repeatedly choose between two alternatives characterized by varying severity levels of the included attributes. The data were analysed using a multinomial logit model; relative attribute importance and maximum acceptable risk measures were calculated. RESULTS: Overall, 231 patients with SSc-ILD completed the DCE. Patients preferred twice-daily oral treatments and 6-12-monthly infusions. Patients' choices were mostly influenced by the risk of GI-AEs or infections. Improvement was more important in respiratory symptoms than in skin tightness. Concerning trade-offs, patients accepted different levels of increase in GI-AE risk: +21% if it reduced the infusions' frequency; +15% if changing to an oral treatment; up to + 37% if it improved breathlessness; and up to + 36% if it reduced the risk of infections. CONCLUSIONS: This is the first study to quantitatively elicit patients' preferences for treatment attributes in SSc-ILD. Patients showed willingness to make trade-offs, providing a firm basis for shared decision-making in clinical practice.
Published: 2022

6. A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.

Author: Saketkoo, Lesley, Saketkoo, Lesley, Frech, Tracy, Varjú, Cecília, Domsic, Robyn, Farrell, Jessica, Gordon, Jessica, Mihai, Carina, Sandorfi, Nora, Shapiro, Lee, Poole, Janet, Volkmann, Elizabeth, Lammi, Monika, McAnally, Kendra, Alexanderson, Helene, Pettersson, Henrik, Hant, Faye, Kuwana, Masataka, Shah, Ami, Smith, Vanessa, Hsu, Vivien, Kowal-Bielecka, Otylia, Assassi, Shervin, Cutolo, Maurizio, Kayser, Cristiane, Shanmugam, Victoria, Vonk, Madelon, Fligelstone, Kim, Baldwin, Nancy, Connolly, Kerri, Ronnow, Anneliese, Toth, Beata, Suave, Maureen, Farrington, Sue, Bernstein, Elana, Crofford, Leslie, Czirják, László, Jensen, Kelly, Hinchclif, Monique, Hudson, Marie, Lammi, Matthew, Mansour, Jennifer, Morgan, Nadia, Mendoza, Fabian, Nikpour, Mandana, Pauling, John, Riemekasten, Gabriela, Russell, Anne-Marie, Scholand, Mary, Seigart, Elise, Rodriguez-Reyna, Tatiana, Hummers, Laura, Walker, Ulrich, Steen, Virginia, Saketkoo, Lesley, Saketkoo, Lesley, Frech, Tracy, Varjú, Cecília, Domsic, Robyn, Farrell, Jessica, Gordon, Jessica, Mihai, Carina, Sandorfi, Nora, Shapiro, Lee, Poole, Janet, Volkmann, Elizabeth, Lammi, Monika, McAnally, Kendra, Alexanderson, Helene, Pettersson, Henrik, Hant, Faye, Kuwana, Masataka, Shah, Ami, Smith, Vanessa, Hsu, Vivien, Kowal-Bielecka, Otylia, Assassi, Shervin, Cutolo, Maurizio, Kayser, Cristiane, Shanmugam, Victoria, Vonk, Madelon, Fligelstone, Kim, Baldwin, Nancy, Connolly, Kerri, Ronnow, Anneliese, Toth, Beata, Suave, Maureen, Farrington, Sue, Bernstein, Elana, Crofford, Leslie, Czirják, László, Jensen, Kelly, Hinchclif, Monique, Hudson, Marie, Lammi, Matthew, Mansour, Jennifer, Morgan, Nadia, Mendoza, Fabian, Nikpour, Mandana, Pauling, John, Riemekasten, Gabriela, Russell, Anne-Marie, Scholand, Mary, Seigart, Elise, Rodriguez-Reyna, Tatiana, Hummers, Laura, Walker, Ulrich, and Steen, Virginia
Abstract: Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to
Published: 2021

7. Natural variability in the disease course of SSc-ILD: implications for treatment.

Author: Vonk, Madelon, Vonk, Madelon, Walker, Ulrich, Volkmann, Elizabeth, Kreuter, Michael, Johnson, Sindhu, Allanore, Yannick, Vonk, Madelon, Vonk, Madelon, Walker, Ulrich, Volkmann, Elizabeth, Kreuter, Michael, Johnson, Sindhu, and Allanore, Yannick
Abstract: Interstitial lung disease (ILD) affects approximately 50% of patients with systemic sclerosis (SSc) and is the leading cause of death in SSc. Our objective was to gain insight into the progression of SSc-associated ILD (SSc-ILD). Using data from longitudinal clinical trials and observational studies, we assessed definitions and patterns of progression, risk factors for progression, and implications for treatment. SSc-ILD progression was commonly defined as exceeding specific thresholds of lung function worsening and/or increasing radiographic involvement. One definition used in several studies is decline in forced vital capacity (FVC) of ≥10%, or ≥5-10% plus a decline in diffusing capacity of the lung for carbon monoxide ≥15%. Based on these criteria, 20-30% of patients in observational cohorts develop progressive ILD, starting early in the disease course and progressing at a highly variable rate.Risk factors such as age, FVC, extent of fibrosis and presence of anti-topoisomerase I antibodies can help predict progression of SSc-ILD, though composite risk scores may offer greater predictive power. Whilst the variability of the disease course in SSc-ILD makes risk stratification of patients challenging, the decision to initiate, change or stop treatment should be based on a combination of the current disease state and the speed of progression.
Published: 2021

8. The burden of systemic sclerosis in Switzerland - the Swiss systemic sclerosis EUSTAR cohort

Author: Hernández, Jasmin, Jordan, Suzana; https://orcid.org/0000-0002-8114-8239, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Iudici, Michele, Hasler, Paul, Ribi, Camillo, Villiger, Peter, Vlachoyiannopoulos, Panayiotis, Vacca, Alessandra, Garzanova, Ludmila, Giollo, Alessandro, Rosato, Edoardo; https://orcid.org/0000-0002-7417-8093, Kötter, Ina, Carreira, Patricia E, Doria, Andrea, Henes, Jörg, Müller-Ladner, Ulf, Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Distler, Jörg H W; https://orcid.org/0000-0001-7408-9333, Gabrielli, Armando, Hoffman-Vold, Anna-Maria, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, The Eustar Collaborators, Hernández, Jasmin, Jordan, Suzana; https://orcid.org/0000-0002-8114-8239, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Iudici, Michele, Hasler, Paul, Ribi, Camillo, Villiger, Peter, Vlachoyiannopoulos, Panayiotis, Vacca, Alessandra, Garzanova, Ludmila, Giollo, Alessandro, Rosato, Edoardo; https://orcid.org/0000-0002-7417-8093, Kötter, Ina, Carreira, Patricia E, Doria, Andrea, Henes, Jörg, Müller-Ladner, Ulf, Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Distler, Jörg H W; https://orcid.org/0000-0001-7408-9333, Gabrielli, Armando, Hoffman-Vold, Anna-Maria, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, and The Eustar Collaborators
Abstract: OBJECTIVES: Characteristics of Swiss patients with systemic sclerosis have not been described so far. The aim of the current study was to identify unmet needs in comparison with other European countries that could inform specific interventions to improve the care of systemic sclerosis patients. METHODS: We analysed Swiss and other European systemic sclerosis patients registered in European Scleroderma Trials And Research (EUSTAR) and the Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) cohort. Demographics, clinical profiles, organ involvement and survival of established, early/mild and very early / very mild systemic sclerosis patients were described and compared between the cohorts. RESULTS: We included 679 Swiss and 8793 European systemic sclerosis patients in the analysis. Over 95% of patients in both cohorts were Caucasian, disease subsets were similar, and no age difference was found. The Swiss cohort had more male patients (25% vs 16% European, p = 0.005) and higher prevalence of early/mild and very early / very mild patients (26.1 vs 8.5% European and 14.9% vs 6.7% European, respectively, both p <0.0001). Disease duration in established systemic sclerosis patients at first presentation was numerically shorter but not significant in the Swiss cohort: 5.0 years (1–12) Swiss vs 6.0 years (2–12) years European, p = 0.055). Despite the earlier referral of Swiss patients to systemic sclerosis expert centres, they showed evidence of more severe disease, particularly in the limited cutaneous systemic sclerosis subset, but no differences in overall survival on longitudinal follow-up were observed. CONCLUSION: This is the first report of the national Swiss EUSTAR cohort. It identifies earlier referral to systemic sclerosis expert centres, before major organ damage occurs, and when outcome can still be modified, as a priority to improve care of patients with systemic sclerosis.
Published: 2021

9. Patient and healthcare professional eHealth literacy and needs for systemic sclerosis support: a mixed methods study

Author: Kocher, Agnes; https://orcid.org/0000-0002-8716-9727, Simon, Michael, Dwyer, Andrew A, Blatter, Catherine, Bogdanovic, Jasmina, Künzler-Heule, Patrizia, Villiger, Peter M, Dan, Diana, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Nicca, Dunja; https://orcid.org/0000-0002-4133-8177, Kocher, Agnes; https://orcid.org/0000-0002-8716-9727, Simon, Michael, Dwyer, Andrew A, Blatter, Catherine, Bogdanovic, Jasmina, Künzler-Heule, Patrizia, Villiger, Peter M, Dan, Diana, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, and Nicca, Dunja; https://orcid.org/0000-0002-4133-8177
Abstract: OBJECTIVES: We engaged patients with systemic sclerosis (SSc) and healthcare professionals to assess electronic health (eHealth) literacy and needs relating to web-based support using internet-based information and communication technologies (ICT). METHODS: We employed an explanatory sequential mixed methods design. First, we conducted a cross-sectional survey in patients (n=101) and professionals (n=47). Next, we conducted three focus groups with patients, family members and professionals (n=17). RESULTS: Of patients, 89.1% used ICT at least weekly for private communication. Patients reported relatively high comprehension of eHealth information ([Formula: see text] =6.7, 95% CI: 6.2 to 7.3, range 1-10), yet were less confident evaluating information reliability ([Formula: see text] =5.8, 95% CI: 5.1 to 6.4) and finding eHealth apps ([Formula: see text] =4.8, 95% CI: 4.2 to 5.4). Patients and professionals reported little experience with web-based self-management support. Focus groups revealed 'considering non-ICT-accessible groups' and 'fitting patients' and professionals' technology' as crucial for acceptability. In relation to understanding/appraising eHealth, participants highlighted that general SSc information is not tailored to individual's disease course. Recommendations included 'providing timely, understandable and safe information' and 'empowering end-users in ICT and health decision-making skills'. Professionals expressed concerns about lacking resources. Patients were concerned about data security and person-centredness. Key eHealth drivers included 'addressing end-user perceptions' and 'putting people at the centre of technology'. CONCLUSIONS: Patients and professionals need education/training to support uptake of eHealth resources. Key elements include guiding patients to timely/reliable information and using eHealth to optimise patient-provider communication. Design that is responsive to end-users' needs and considers individuals with limited eHealth
Published: 2021

10. Discontinuation of biologic DMARDs in a real-world population of patients with rheumatoid arthritis in remission: outcome and risk factors

Author: Arnold, Simone, Jaeger, Veronika K, Scherer, Almut, Ciurea, Adrian, Walker, Ulrich A, Kyburz, Diego, Arnold, Simone, Jaeger, Veronika K, Scherer, Almut, Ciurea, Adrian, Walker, Ulrich A, and Kyburz, Diego
Abstract: OBJECTIVES Data from randomized controlled trials have shown the feasibility of discontinuation of bDMARD therapy in patients with RA that have reached remission. Criteria for selecting patients that are likely to remain in remission are still incompletely defined.We aimed to identify predictors of successful discontinuation of bDMARD therapy in the Swiss Clinical Quality Management (SCQM) registry, a real-world cohort of RA patients. METHODS RA patients in DAS28-ESR remission who stopped bDMARD/tsDMARD treatment were included. Loss of remission was defined as a DAS28-ESR > 2.6 or restart of a bDMARD/tsDMARD. Time to loss of remission was the main outcome. Kaplan-Meier methods were applied and cox regression was used for multivariable analyses adjusting for confounding factors. Missing data were imputed using multiple imputation. RESULTS 318 patients in a bDMARD/tsDMARD-free remission were followed between 1997 and 2017. 241 patients (76%) lost remission after a median time of 0.9 years (95%CI 0.7-1.0). The time to loss of remission was shorter in women, in patients with a longer disease duration >4yrs and in patients who did not meet CDAI remission criteria at baseline. Remission was longer in patients with csDMARD therapy during b/tsDMARD free remission (HR 0.8, p= 0.05, 95%CI 0.6-1.0). CONCLUSION In a real-world patient population the majority of patients who discontinued b/tsDMARD treatment lost remission within <1 year. Our study confirms that fulfilment of more rigorous remission criteria and csDMARD treatment increases the chance of maintaining b/tsDMARD free remission.
Published: 2021

11. Impact of assessing patient-reported outcomes with mobile apps on patient-provider interaction

Author: Shaw, Yomei; https://orcid.org/0000-0001-7048-8687, Courvoisier, Delphine S, Scherer, Almut, Ciurea, Adrian; https://orcid.org/0000-0002-7870-7132, Lehmann, Thomas, Jaeger, Veronika K, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Finckh, Axel; https://orcid.org/0000-0002-1210-4347, Shaw, Yomei; https://orcid.org/0000-0001-7048-8687, Courvoisier, Delphine S, Scherer, Almut, Ciurea, Adrian; https://orcid.org/0000-0002-7870-7132, Lehmann, Thomas, Jaeger, Veronika K, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, and Finckh, Axel; https://orcid.org/0000-0002-1210-4347
Abstract: OBJECTIVE To explore the effect of apps measuring patient-reported outcomes (PROs) on patient-provider interaction in the rheumatic diseases in an observational setting. METHODS Patients in the Swiss Clinical Quality Management in Rheumatic Diseases Registry were offered mobile apps (iDialog and COmPASS) to track disease status between rheumatology visits using validated PROs (Rheumatoid Arthritis Disease Activity Index-5 score, Bath Ankylosing Spondylitis Disease Activity Index score, Routine Assessment of Patient Index Data-3 score and Visual Analogue Scale score for pain, disease activity and skin symptoms). We assessed two aspects of patient-provider interaction: shared decision making (SDM) and physician awareness of disease fluctuations. We used logistic regressions to compare outcomes among patients who (1) used an app and discussed app data with their physician (app+discussion group), (2) used an app without discussing the data (app-only group) or (3) did not use any app (non-app users). RESULTS 2111 patients were analysed, including 1799 non-app users, 150 app-only users and 162 app+discussion users (43% male; with 902 patients with rheumatoid arthritis, 766 patients with axial spondyloarthritis and 443 patients with psoriatic arthritis). App users were younger than non-app users (mean age of 47 vs 51 years, p<0.001). Compared with non-app users, the app+discussion group rated their rheumatologist more highly in SDM (OR 1.7, 95% CI 1.1 to 2.4) and physician awareness of disease fluctuations (OR 2.0, 95% CI 1.3 to 3.1). This improvement was absent in the app-only group. CONCLUSION App users who discussed app data with their rheumatologist reported more favourably on patient-provider interactions than app users who did not and non-app users. Apps measuring PROs may contribute little to patient-provider interactions without integration of app data into care processes.
Published: 2021

12. A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: The UPSIDE study protocol

Author: MS Reumatologie/Immunologie/Infectie, Infection & Immunity, MS Hematologie, Cancer, Lab Reumatologie/Klinische Immunologie, Regenerative Medicine and Stem Cells, JC onderzoeksprogramma Methodologie, Spierings, Julia, van Rhenen, Anna, Welsing, Paco Mw, Marijnissen, Anne Ca, De Langhe, Ellen, Del Papa, Nicoletta, Dierickx, Daan, Gheorghe, Karina R, Henes, Joerg, Hesselstrand, Roger, Kerre, Tessa, Ljungman, Per, van de Loosdrecht, Arjan A, Marijt, Erik Waf, Mayer, Miro, Schmalzing, Marc, Schroers, Roland, Smith, Vanessa, Voll, Reinhard E, Vonk, Madelon C, Voskuyl, Alexandre E, de Vries-Bouwstra, Jeska K, Walker, Ulrich A, Wuttge, Dirk M, van Laar, Jacob M, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, MS Hematologie, Cancer, Lab Reumatologie/Klinische Immunologie, Regenerative Medicine and Stem Cells, JC onderzoeksprogramma Methodologie, Spierings, Julia, van Rhenen, Anna, Welsing, Paco Mw, Marijnissen, Anne Ca, De Langhe, Ellen, Del Papa, Nicoletta, Dierickx, Daan, Gheorghe, Karina R, Henes, Joerg, Hesselstrand, Roger, Kerre, Tessa, Ljungman, Per, van de Loosdrecht, Arjan A, Marijt, Erik Waf, Mayer, Miro, Schmalzing, Marc, Schroers, Roland, Smith, Vanessa, Voll, Reinhard E, Vonk, Madelon C, Voskuyl, Alexandre E, de Vries-Bouwstra, Jeska K, Walker, Ulrich A, Wuttge, Dirk M, and van Laar, Jacob M
Published: 2021

13. Tocilizumab in systemic sclerosis:a randomised, double-blind, placebo-controlled, phase 3 trial

Author: Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, Steen, Virginia D., Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, and Steen, Virginia D.
Abstract: Background: A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in a phase 3 trial to investigate the safety and efficacy of tocilizumab, an anti-interleukin-6 receptor antibody, in the treatment of systemic sclerosis. Methods: In this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial, participants were recruited from 75 sites in 20 countries across Europe, North America, Latin America, and Japan. Adults with diffuse cutaneous systemic sclerosis for 60 months or less and a modified Rodnan skin score (mRSS) of 10–35 at screening were randomly assigned (1:1) with a voice-web-response system to receive subcutaneous tocilizumab 162 mg or placebo weekly for 48 weeks, stratified by IL-6 levels; participants and investigators were masked to treatment group. The primary endpoint was the difference in change from baseline to week 48 in mRSS. Percentage of predicted forced vital capacity (FVC% predicted) at week 48, time to treatment failure, and patient-reported and physician-reported outcomes were secondary endpoints. This trial is registered with ClinicalTrials.gov (number NCT02453256) and is closed to accrual. Findings: Between Nov 20, 2015, and Feb 14, 2017, 210 individuals were randomly assigned to receive tocilizumab (n=104) or placebo (n=106). In the intention-to-treat population, least squares mean [LSM] change from baseline to week 48 in mRSS was −6·14 for tocilizumab and −4·41 for placebo (adjusted difference −1·73 [95% CI −3·78 to 0·32]; p=0·10). The shift in distribution of change from baseline in FVC% predicted at week 48 favoured tocilizumab (van Elteren nominal p=0·002 vs placebo), with a difference in LSM of 4·2 (95% CI 2·0–6·4; nominal p=0·0002), as did time to treatment failure (hazard ratio 0·63 [95% CI 0·37–1·06]; nominal p=0·08). Change in LSM from baseline to week 48 in Health Assessment Ques
Published: 2020

14. Tocilizumab in systemic sclerosis:a randomised, double-blind, placebo-controlled, phase 3 trial

Author: Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, Steen, Virginia D., Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, and Steen, Virginia D.
Abstract: Background: A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in a phase 3 trial to investigate the safety and efficacy of tocilizumab, an anti-interleukin-6 receptor antibody, in the treatment of systemic sclerosis. Methods: In this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial, participants were recruited from 75 sites in 20 countries across Europe, North America, Latin America, and Japan. Adults with diffuse cutaneous systemic sclerosis for 60 months or less and a modified Rodnan skin score (mRSS) of 10–35 at screening were randomly assigned (1:1) with a voice-web-response system to receive subcutaneous tocilizumab 162 mg or placebo weekly for 48 weeks, stratified by IL-6 levels; participants and investigators were masked to treatment group. The primary endpoint was the difference in change from baseline to week 48 in mRSS. Percentage of predicted forced vital capacity (FVC% predicted) at week 48, time to treatment failure, and patient-reported and physician-reported outcomes were secondary endpoints. This trial is registered with ClinicalTrials.gov (number NCT02453256) and is closed to accrual. Findings: Between Nov 20, 2015, and Feb 14, 2017, 210 individuals were randomly assigned to receive tocilizumab (n=104) or placebo (n=106). In the intention-to-treat population, least squares mean [LSM] change from baseline to week 48 in mRSS was −6·14 for tocilizumab and −4·41 for placebo (adjusted difference −1·73 [95% CI −3·78 to 0·32]; p=0·10). The shift in distribution of change from baseline in FVC% predicted at week 48 favoured tocilizumab (van Elteren nominal p=0·002 vs placebo), with a difference in LSM of 4·2 (95% CI 2·0–6·4; nominal p=0·0002), as did time to treatment failure (hazard ratio 0·63 [95% CI 0·37–1·06]; nominal p=0·08). Change in LSM from baseline to week 48 in Health Assessment Ques
Published: 2020

15. Travel patterns, risk behaviour and health problems of travellers with rheumatic diseases compared to controls: A multi-centre, observational study

Author: Schmid, Nathan, Ciurea, Adrian, Gabay, Cem, Hasler, Paul, Fehr, Jan, Müller, Rüdiger, Villiger, Peter, Walker, Ulrich, Hatz, Christoph, Bühler, Silja, Schmid, Nathan, Ciurea, Adrian, Gabay, Cem, Hasler, Paul, Fehr, Jan, Müller, Rüdiger, Villiger, Peter, Walker, Ulrich, Hatz, Christoph, and Bühler, Silja
Abstract: BACKGROUND Patients with chronic conditions travel around the world more than ever. Only few studies have examined travel patterns and health outcomes of patients with rheumatic diseases during international travel. METHOD We conducted a multi-centre prospective cohort study in Switzerland, in which we studied the immunogenicity and safety of vaccinations in patients with rheumatic diseases and travellers without rheumatic diseases (controls). Participants who travelled internationally received questionnaires 1 and 13 weeks post-travel. We compared travel patterns, risk behaviours, and travel-associated problems during and after the trips in both groups. RESULTS 274 participants returned post-travel questionnaires (65 rheumatic patients, 209 controls). Controls more frequently travelled to subtropical/tropical destinations and stayed longer abroad. 64% of all participants experienced health problems during travel (74% rheumatic patients vs. 62% controls, P = 0.11). Pre-travel, patients reported a higher susceptibility to gastrointestinal infections. During travel, a higher percentage of rheumatic patients cancelled the day programme due to health problems (13% vs. 4%, P = 0.024). The main problems in rheumatic patients occurred due to the underlying rheumatic diseases, or were of psychological nature. Although not statistically significant, infectious disease symptoms (rhinitis, cough) occurred more frequently in controls. When only considering subtropical/tropical destinations, rheumatic patients more frequently had gastrointestinal problems during travel - and skin infections after the trip. CONCLUSIONS This study does not support the notion that patients with rheumatic diseases should avoid international travel for an increased risk of infections. In patients with subtropical/tropical destinations, however, gastrointestinal problems may be increased during travel - and skin infections post-travel.
Published: 2020

16. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

Author: Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, and Puttini, Piercarlo Sarzi
Abstract: Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) >= 7, valid mRSS at 12 +/- 3 months after baseline and >= 1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and >= 25% from baseline to 12 +/- 3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline >= 10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (<= 22/51) or short disease duration (<= 15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline >= 10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). Conclusions Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.
Published: 2019

17. Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Author: Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg H. W., Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C., Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M., Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg H. W., Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C., Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M., Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, and Poindron, Vincent
Abstract: Objective Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. Methods A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. Results Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. Conclusion Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis.
Published: 2019

18. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author: Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan Jose, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I., Lescoat, Alain, Siegert, Elise, Castellvi, Ivan, Airo, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, Lopez-Longo, F. J., Distler, Joerg H. W., Hunzelmann, Nicolas, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M., Pozzi, Maria R., Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, Guiducci, Serena, Walker, Ulrich A., Kyburz, Diego, Lapadula, Giovanni, Maurer, Britta, Jordan, Suzana, Dobrota, Rucsandra, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Sulli, Alberto, Cutolo, Maurizio, Cuomo, Giovanna, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis G., Montecucco, Carlo Maurizio, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Wollheim, Frank, Scheja, Agneta, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Trotta, F., Lo Monaco, Andrea, Pellerito, Raffaele, Mauriziano, Ospedale, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jorg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Da Silva, Jose Antonio Pereira, Salvador, Maria Joao, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Tikly, Mohammed, Denisov, Lev N., Herrick, Ariane, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriela, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Sifuentes W. A., Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Launay, David, Valesini, Guido, Ionescu, Ruxandra Maria, Groseanu, Laura, Opris, Daniela, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Milicescu, Mihaela, Sunderkotter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Melsens, Karin, Cantatore, Francesco P., Corrado, Ada, Iversen, Line, von Muhlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Eyerich, Kilian, Hein, Rudiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Gunther, Claudia, Westhovens, Rene, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Granel, Brigitte, Muller, Carolina de Souza, Radominski, Sebastiao C., Azevedo, Valderilio F., Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stebbings, Simon, Highton, John, Chapman, Peter, O'Donnell, John, Stamp, Lisa, Doube, Alan, Solanki, Kamal, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Furst, Daniel, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, Hassanien, Manal, Lefebvre, Paloma Garcia de la Pena, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Chatelus, Emmanuel, Sibilia, Jean, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Lasky, Joseph A., Cosentino, Vanesa, Kerzberg, Eduardo, Montoya, Fabiana, Bianchi, Washington, Carneiro, Sueli, Maretti, Giselle Baptista, Bianchi, Dante Valdetaro, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Kahl, Sarah, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chotchaeva, Fatima, Mukhin, Nikolay A., Moiseev, Sergey, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan Jose, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I., Lescoat, Alain, Siegert, Elise, Castellvi, Ivan, Airo, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, Lopez-Longo, F. J., Distler, Joerg H. W., Hunzelmann, Nicolas, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M., Pozzi, Maria R., Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, Guiducci, Serena, Walker, Ulrich A., Kyburz, Diego, Lapadula, Giovanni, Maurer, Britta, Jordan, Suzana, Dobrota, Rucsandra, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Sulli, Alberto, Cutolo, Maurizio, Cuomo, Giovanna, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis G., Montecucco, Carlo Maurizio, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Wollheim, Frank, Scheja, Agneta, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Trotta, F., Lo Monaco, Andrea, Pellerito, Raffaele, Mauriziano, Ospedale, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jorg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Da Silva, Jose Antonio Pereira, Salvador, Maria Joao, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Tikly, Mohammed, Denisov, Lev N., Herrick, Ariane, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriela, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Sifuentes W. A., Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Launay, David, Valesini, Guido, Ionescu, Ruxandra Maria, Groseanu, Laura, Opris, Daniela, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Milicescu, Mihaela, Sunderkotter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Melsens, Karin, Cantatore, Francesco P., Corrado, Ada, Iversen, Line, von Muhlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Eyerich, Kilian, Hein, Rudiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Gunther, Claudia, Westhovens, Rene, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Granel, Brigitte, Muller, Carolina de Souza, Radominski, Sebastiao C., Azevedo, Valderilio F., Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stebbings, Simon, Highton, John, Chapman, Peter, O'Donnell, John, Stamp, Lisa, Doube, Alan, Solanki, Kamal, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Furst, Daniel, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, Hassanien, Manal, Lefebvre, Paloma Garcia de la Pena, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Chatelus, Emmanuel, Sibilia, Jean, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Lasky, Joseph A., Cosentino, Vanesa, Kerzberg, Eduardo, Montoya, Fabiana, Bianchi, Washington, Carneiro, Sueli, Maretti, Giselle Baptista, Bianchi, Dante Valdetaro, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Kahl, Sarah, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chotchaeva, Fatima, Mukhin, Nikolay A., and Moiseev, Sergey
Abstract: Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.
Published: 2019

19. Fast track algorithm: How to differentiate a 'scleroderma pattern' from a 'non-scleroderma pattern'

Author: Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Vanhaecke, Amber, Herrick, Ariane L, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Guerra, Miguel G, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Deschepper, Ellen, Foeldvari, Ivan, Gutierrez, Marwin, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Ingegnoli, Francesca, Kubo, Satoshi, Müller-Ladner, Ulf, Riccieri, Valeria, Sulli, Alberto, van Laar, Jaap M, Vonk, Madelon C; https://orcid.org/0000-0002-2266-9907, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Cutolo, Maurizio; https://orcid.org/0000-0002-5396-0932, EULAR Study Group on Microcirculation in Rheumatic Diseases, Smith, Vanessa; https://orcid.org/0000-0001-6271-7945, Vanhaecke, Amber, Herrick, Ariane L, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Guerra, Miguel G, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Deschepper, Ellen, Foeldvari, Ivan, Gutierrez, Marwin, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Ingegnoli, Francesca, Kubo, Satoshi, Müller-Ladner, Ulf, Riccieri, Valeria, Sulli, Alberto, van Laar, Jaap M, Vonk, Madelon C; https://orcid.org/0000-0002-2266-9907, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Cutolo, Maurizio; https://orcid.org/0000-0002-5396-0932, and EULAR Study Group on Microcirculation in Rheumatic Diseases
Abstract: OBJECTIVES This study was designed to propose a simple "Fast Track algorithm" for capillaroscopists of any level of experience to differentiate "scleroderma patterns" from "non-scleroderma patterns" on capillaroscopy and to assess its inter-rater reliability. METHODS Based on existing definitions to categorise capillaroscopic images as "scleroderma patterns" and taking into account the real life variability of capillaroscopic images described standardly according to the European League Against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases, a fast track decision tree, the "Fast Track algorithm" was created by the principal expert (VS) to facilitate swift categorisation of an image as "non-scleroderma pattern (category 1)" or "scleroderma pattern (category 2)". Mean inter-rater reliability between all raters (experts/attendees) of the 8th EULAR course on capillaroscopy in Rheumatic Diseases (Genoa, 2018) and, as external validation, of the 8th European Scleroderma Trials and Research group (EUSTAR) course on systemic sclerosis (SSc) (Nijmegen, 2019) versus the principal expert, as well as reliability between the rater pairs themselves was assessed by mean Cohen's and Light's kappa coefficients. RESULTS Mean Cohen's kappa was 1/0.96 (95% CI 0.95-0.98) for the 6 experts/135 attendees of the 8th EULAR capillaroscopy course and 1/0.94 (95% CI 0.92-0.96) for the 3 experts/85 attendees of the 8th EUSTAR SSc course. Light's kappa was 1/0.92 at the 8th EULAR capillaroscopy course, and 1/0.87 at the 8th EUSTAR SSc course. CONCLUSION For the first time, a clinical expert based fast track decision algorithm has been developed to differentiate a "non-scleroderma" from a "scleroderma pattern" on capillaroscopic images, demonstrating excellent reliability when applied by capillaroscopists with varying levels of expertise versus the principal expert and corroborated with external validation.
Published: 2019

20. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author: Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme; https://orcid.org/0000-0002-2463-218X, Cometi, L, Czirják, László, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Müller-Ladner, Ulf, Del Galdo, Francesco; https://orcid.org/0000-0002-8528-2283, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, EUSTAR co-workers, Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme; https://orcid.org/0000-0002-2463-218X, Cometi, L, Czirják, László, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Müller-Ladner, Ulf, Del Galdo, Francesco; https://orcid.org/0000-0002-8528-2283, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, and EUSTAR co-workers
Abstract: BACKGROUND A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc). METHODS DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked. RESULTS A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU. CONCLUSIONS For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated
Published: 2019

21. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

Author: Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, and Puttini, Piercarlo Sarzi
Abstract: Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) >= 7, valid mRSS at 12 +/- 3 months after baseline and >= 1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and >= 25% from baseline to 12 +/- 3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline >= 10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (<= 22/51) or short disease duration (<= 15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline >= 10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). Conclusions Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.
Published: 2019

22. Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Author: Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg H. W., Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C., Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M., Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg H. W., Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C., Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M., Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, and Poindron, Vincent
Abstract: Objective Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. Methods A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. Results Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. Conclusion Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis.
Published: 2019

23. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author: Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan Jose, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I., Lescoat, Alain, Siegert, Elise, Castellvi, Ivan, Airo, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, Lopez-Longo, F. J., Distler, Joerg H. W., Hunzelmann, Nicolas, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M., Pozzi, Maria R., Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, Guiducci, Serena, Walker, Ulrich A., Kyburz, Diego, Lapadula, Giovanni, Maurer, Britta, Jordan, Suzana, Dobrota, Rucsandra, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Sulli, Alberto, Cutolo, Maurizio, Cuomo, Giovanna, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis G., Montecucco, Carlo Maurizio, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Wollheim, Frank, Scheja, Agneta, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Trotta, F., Lo Monaco, Andrea, Pellerito, Raffaele, Mauriziano, Ospedale, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jorg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Da Silva, Jose Antonio Pereira, Salvador, Maria Joao, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Tikly, Mohammed, Denisov, Lev N., Herrick, Ariane, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriela, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Sifuentes W. A., Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Launay, David, Valesini, Guido, Ionescu, Ruxandra Maria, Groseanu, Laura, Opris, Daniela, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Milicescu, Mihaela, Sunderkotter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Melsens, Karin, Cantatore, Francesco P., Corrado, Ada, Iversen, Line, von Muhlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Eyerich, Kilian, Hein, Rudiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Gunther, Claudia, Westhovens, Rene, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Granel, Brigitte, Muller, Carolina de Souza, Radominski, Sebastiao C., Azevedo, Valderilio F., Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stebbings, Simon, Highton, John, Chapman, Peter, O'Donnell, John, Stamp, Lisa, Doube, Alan, Solanki, Kamal, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Furst, Daniel, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, Hassanien, Manal, Lefebvre, Paloma Garcia de la Pena, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Chatelus, Emmanuel, Sibilia, Jean, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Lasky, Joseph A., Cosentino, Vanesa, Kerzberg, Eduardo, Montoya, Fabiana, Bianchi, Washington, Carneiro, Sueli, Maretti, Giselle Baptista, Bianchi, Dante Valdetaro, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Kahl, Sarah, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chotchaeva, Fatima, Mukhin, Nikolay A., Moiseev, Sergey, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan Jose, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I., Lescoat, Alain, Siegert, Elise, Castellvi, Ivan, Airo, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, Lopez-Longo, F. J., Distler, Joerg H. W., Hunzelmann, Nicolas, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M., Pozzi, Maria R., Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, Guiducci, Serena, Walker, Ulrich A., Kyburz, Diego, Lapadula, Giovanni, Maurer, Britta, Jordan, Suzana, Dobrota, Rucsandra, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Sulli, Alberto, Cutolo, Maurizio, Cuomo, Giovanna, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis G., Montecucco, Carlo Maurizio, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Wollheim, Frank, Scheja, Agneta, Wuttge, Dirk M., Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Trotta, F., Lo Monaco, Andrea, Pellerito, Raffaele, Mauriziano, Ospedale, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jorg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Da Silva, Jose Antonio Pereira, Salvador, Maria Joao, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Tikly, Mohammed, Denisov, Lev N., Herrick, Ariane, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriela, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Sifuentes W. A., Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Launay, David, Valesini, Guido, Ionescu, Ruxandra Maria, Groseanu, Laura, Opris, Daniela, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Milicescu, Mihaela, Sunderkotter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Melsens, Karin, Cantatore, Francesco P., Corrado, Ada, Iversen, Line, von Muhlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Eyerich, Kilian, Hein, Rudiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A., Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Gunther, Claudia, Westhovens, Rene, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Granel, Brigitte, Muller, Carolina de Souza, Radominski, Sebastiao C., Azevedo, Valderilio F., Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Yoshinari, Natalino H., Marangoni, Roberta G., Martin, Patricia, Fuocco, Luiza, Stebbings, Simon, Highton, John, Chapman, Peter, O'Donnell, John, Stamp, Lisa, Doube, Alan, Solanki, Kamal, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Furst, Daniel, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, Hassanien, Manal, Lefebvre, Paloma Garcia de la Pena, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Chatelus, Emmanuel, Sibilia, Jean, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Lasky, Joseph A., Cosentino, Vanesa, Kerzberg, Eduardo, Montoya, Fabiana, Bianchi, Washington, Carneiro, Sueli, Maretti, Giselle Baptista, Bianchi, Dante Valdetaro, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Kahl, Sarah, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chotchaeva, Fatima, Mukhin, Nikolay A., and Moiseev, Sergey
Abstract: Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.
Published: 2019

24. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group

Author: Jaeger, Veronika K., Distler, Oliver, Maurer, Britta, Czirjak, Laszlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Doerte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Toth, Beata Garay, Fankhauser, Beat, Umbricht, Jorg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, de la Pena Lefebvre, Paloma Garciae, Gabrielli, Armando, Krummel-Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Mueller-Ladner, Ulf, Walker, Ulrich A., Jaeger, Veronika K., Distler, Oliver, Maurer, Britta, Czirjak, Laszlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Doerte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Toth, Beata Garay, Fankhauser, Beat, Umbricht, Jorg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, de la Pena Lefebvre, Paloma Garciae, Gabrielli, Armando, Krummel-Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Mueller-Ladner, Ulf, and Walker, Ulrich A.
Abstract: The multisystem manifestations of SSc can greatly impact patients' quality of life. The aim of this study was to identify factors associated with disability in SSc. SSc patients from the prospective DeSScipher cohort who had completed the scleroderma health assessment questionnaire (SHAQ), a disability score that combines the health assessment questionnaire and five visual analogue scales, were included in this analysis. The effect of factors possibly associated with disability was analysed with multiple linear regressions. The mean SHAQ and HAQ scores of the 944 patients included were 0.87 (s.d. = 0.66) and 0.92 (s.d. = 0.78); 59% of the patients were in the mild to moderate difficulty SHAQ category (0 a (c) 1/2 SHAQ < 1), 34% in the moderate to severe disability category (1 a (c) 1/2 SHAQ < 2) and 7% in the severe to very severe disability category (2 a (c) 1/2 SHAQ a (c) 1/2 3). The means of the visual analogue scales scores were in order of magnitude: overall disease severity (37 mm), RP (31 mm), pulmonary symptoms (24 mm), gastrointestinal symptoms (20 mm) and digital ulcers (19 mm). In multiple regression, the main factors associated with high SHAQ scores were the presence of dyspnoea [modified New York Heart Association (NYHA) class IV (regression coefficient B = 0.62), modified NYHA class III (B = 0.53) and modified NYHA class II (B = 0.21; all modified NYHA class I)], FM (B = 0.37), muscle weakness (B = 0.27), digital ulcers (B = 0.20) and gastrointestinal symptoms (oesophageal symptoms, B = 0.16; stomach symptoms, B = 0.15; intestinal symptoms, B = 0.15). SSc patients perceive dyspnoea, pain, digital ulcers, muscle weakness and gastrointestinal symptoms as the main factors driving their level of disability, unlike physicians who emphasize objective measures of disability.
Published: 2018

25. Prospective evaluation of the capillaroscopic skin ulcer risk index in systemic sclerosis patients in clinical practice: a longitudinal, multicentre study

Author: Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Jaeger, Veronika K, Bruppacher, Katharina M, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Arlettaz, Lionel, Banyai, Martin, Beron, Jörg, Chizzolini, Carlo, Groechenig, Ernst, Mueller, Rüdiger B, Spertini, François, Villiger, Peter M, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Jaeger, Veronika K, Bruppacher, Katharina M, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Arlettaz, Lionel, Banyai, Martin, Beron, Jörg, Chizzolini, Carlo, Groechenig, Ernst, Mueller, Rüdiger B, Spertini, François, Villiger, Peter M, and Distler, Oliver; https://orcid.org/0000-0002-0546-8310
Abstract: BACKGROUND Nailfold capillaroscopy (NC) is an important tool for the diagnosis of systemic sclerosis (SSc). The capillaroscopic skin ulcer risk index (CSURI) was suggested to identify patients at risk of developing digital ulcers (DUs). This study aims to assess the reliability of the CSURI across assessors, the CSURI change during follow-up and the value of the CSURI in predicting new DUs. METHODS This multicentre, longitudinal study included SSc patients with a history of DUs. NC images of all eight fingers were obtained at baseline and follow-up and were separately analysed by two trained assessors. RESULTS Sixty-one patients were included (median observation time 1.0 year). In about 40% of patients (assessor 1, n = 24, 39%; assessor 2, n = 26, 43%) no megacapillary was detected in any of the baseline or follow-up images; hence the CSURI could not be calculated. In those 34 patients in whom CSURI scores were available from both assessors (26% male; median age 57 years) the median baseline CSURI was 5.3 according to assessor 1 (IQR 2.6-16.3), increasing to 5.9 (IQR 1.3-12.0) at follow-up. According to assessor 2, the CSURI diminished from 6.4 (IQR 2.4-12.5) to 5.0 (IQR 1.7-10.0). The ability of a CSURI ≥ 2.96 category to predict new DUs was low (for both assessors, positive predictive value 38% and negative predictive value 50%) and the inter-assessor agreements for CSURI categories were fair to moderate. CONCLUSIONS In this study, around 40% of patients could not be evaluated with the CSURI due to the absence of megacapillaries. Clinical decisions based on the CSURI should be made with caution. TRIAL REGISTRATION Current Controlled Trials, ISRCTN04371709 . Registered on 18 March 2011.
Published: 2018

26. Haematopoietic stem cell transplantation in systemic sclerosis

Author: Walker, Ulrich A, Saketkoo, Lesley Ann, Distler, Oliver, Walker, Ulrich A, Saketkoo, Lesley Ann, and Distler, Oliver
Abstract: Three randomised controlled trials of haematopoietic stem cell transplantation (HSCT) in systemic sclerosis (SSc) demonstrated long-term survival benefits, induction of clinically meaningful, sustained improvement of forced vital capacity with improvements in skin thickening, vasculopathy and health-related quality of life, in contrast to a clinical decline in standard of care control groups. These benefits, however, must be weighed against the increased risk of transplant-related mortality. Further, with disease progression, severe extensive internal organ involvement and damage ensues, constituting an exclusion criterion for safety reasons, leaving a limited window whereby patients with SSc are eligible for HSCT. Although autologous HSCT offers the possibility of drug-free remission, relapse can occur, requiring re-initiation of disease modifying antirheumatic drugs. HSCT is also associated with secondary autoimmune diseases and gonadal failure. HSCT should be proposed for carefully selected patients with early rapidly progressive diffuse SSc whose clinical picture portends a poor prognosis for survival, but yet lacks advanced organ involvement.
Published: 2018

27. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group

Author: Jaeger, Veronika K., Distler, Oliver, Maurer, Britta, Czirjak, Laszlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Doerte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Toth, Beata Garay, Fankhauser, Beat, Umbricht, Jorg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, de la Pena Lefebvre, Paloma Garciae, Gabrielli, Armando, Krummel-Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Mueller-Ladner, Ulf, Walker, Ulrich A., Jaeger, Veronika K., Distler, Oliver, Maurer, Britta, Czirjak, Laszlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Doerte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Toth, Beata Garay, Fankhauser, Beat, Umbricht, Jorg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, de la Pena Lefebvre, Paloma Garciae, Gabrielli, Armando, Krummel-Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Mueller-Ladner, Ulf, and Walker, Ulrich A.
Abstract: The multisystem manifestations of SSc can greatly impact patients' quality of life. The aim of this study was to identify factors associated with disability in SSc. SSc patients from the prospective DeSScipher cohort who had completed the scleroderma health assessment questionnaire (SHAQ), a disability score that combines the health assessment questionnaire and five visual analogue scales, were included in this analysis. The effect of factors possibly associated with disability was analysed with multiple linear regressions. The mean SHAQ and HAQ scores of the 944 patients included were 0.87 (s.d. = 0.66) and 0.92 (s.d. = 0.78); 59% of the patients were in the mild to moderate difficulty SHAQ category (0 a (c) 1/2 SHAQ < 1), 34% in the moderate to severe disability category (1 a (c) 1/2 SHAQ < 2) and 7% in the severe to very severe disability category (2 a (c) 1/2 SHAQ a (c) 1/2 3). The means of the visual analogue scales scores were in order of magnitude: overall disease severity (37 mm), RP (31 mm), pulmonary symptoms (24 mm), gastrointestinal symptoms (20 mm) and digital ulcers (19 mm). In multiple regression, the main factors associated with high SHAQ scores were the presence of dyspnoea [modified New York Heart Association (NYHA) class IV (regression coefficient B = 0.62), modified NYHA class III (B = 0.53) and modified NYHA class II (B = 0.21; all modified NYHA class I)], FM (B = 0.37), muscle weakness (B = 0.27), digital ulcers (B = 0.20) and gastrointestinal symptoms (oesophageal symptoms, B = 0.16; stomach symptoms, B = 0.15; intestinal symptoms, B = 0.15). SSc patients perceive dyspnoea, pain, digital ulcers, muscle weakness and gastrointestinal symptoms as the main factors driving their level of disability, unlike physicians who emphasize objective measures of disability.
Published: 2018

28. The impact of 18F-FDG PET on the management of patients with suspected large vessel vasculitis

Author: Fuchs, Martin, Briel, Matthias, Daikeler, Thomas, Walker, Ulrich, Rasch, Helmut, Berg, Scott, Ng, Quinn, Raatz, Heike, Jayne, David, Kötter, Ina, Blockmans, Daniel, Cid, Maria, Prieto-González, Sergio, Lamprecht, Peter, Salvarani, Carlo, Karageorgaki, Zaharenia, Watts, Richard, Luqmani, Raashid, Müller-Brand, Jan, Tyndall, Alan, Walter, Martin, Fuchs, Martin, Briel, Matthias, Daikeler, Thomas, Walker, Ulrich, Rasch, Helmut, Berg, Scott, Ng, Quinn, Raatz, Heike, Jayne, David, Kötter, Ina, Blockmans, Daniel, Cid, Maria, Prieto-González, Sergio, Lamprecht, Peter, Salvarani, Carlo, Karageorgaki, Zaharenia, Watts, Richard, Luqmani, Raashid, Müller-Brand, Jan, Tyndall, Alan, and Walter, Martin
Abstract: Purpose: We aimed to assess the impact of 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) on the management of patients with suspected large vessel vasculitis. Methods: An international expert panel determined diagnoses and clinical management in patients with suspected large vessel vasculitis, with and without the results of 18F-FDG PET, respectively. The accuracy of the clinical diagnosis and the resulting clinical management with and without the 18F-FDG PET results were compared using logistic regression models. Results: The analysis included 30 patients referred to a tertiary care centre with large vessel vasculitis and 31 controls. 18F-FDG PET had an overall sensitivity of 73.3% [95% confidence interval (CI) 54.1-87.7%], a specificity of 83.9% (95% CI 66.3-94.5%), a positive predictive value of 81.5% (95% CI 61.9-93.7%) and a negative predictive value of 76.5% (95% CI 58.8-89.3%). The diagnostic accuracy of 18F-FDG PET was higher in patients not receiving immunosuppressive drugs (93.3 vs 64.5%, p = 0.006). Taken in context with other available diagnostic modalities, the addition of 18F-FDG PET increased the clinical diagnostic accuracy from 54.1 to 70.5% (p = 0.04). The addition of 18F-FDG PET increased the number of indicated biopsies from 22 of 61 patients (36.1%) to 25 of 61 patients (41.0%) and changed the treatment recommendation in 8 of 30 patients (26.7%) not receiving immunosuppressive medication and in 7 of 31 patients (22.6%) receiving immunosuppressive medication. Conclusion: 18F-FDG PET is a sensitive and specific imaging tool for large vessel vasculitis, especially when performed in patients not receiving immunosuppressive drugs. It increases the overall diagnostic accuracy and has an impact on the clinical management in a significant proportion of patients
Published: 2018

29. Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group

Author: Adler, Sabine, Huscher, Dörte, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Czirják, László, DelGaldo, Francesco, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, Mueller-Ladner, Ulf, Tarner, Ingo-Helmut, Valentini, Gabriele, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Villiger, Peter M, Riemekasten, Gabriela, Adler, Sabine, Huscher, Dörte, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Czirják, László, DelGaldo, Francesco, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, Mueller-Ladner, Ulf, Tarner, Ingo-Helmut, Valentini, Gabriele, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Villiger, Peter M, and Riemekasten, Gabriela
Abstract: BACKGROUND: Interstitial lung disease in systemic sclerosis (SSc-ILD) is a major cause of SSc-related death. Imunosuppressive treatment (IS) is used in patients with SSc for various organ manifestations mainly to ameliorate progression of SSc-ILD. Data on everyday IS prescription patterns and clinical courses of lung function during and after therapy are scarce. METHODS: We analysed patients fulfilling American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) 2013 criteria for SSc-ILD and at least one report of IS. Types of IS, pulmonary function tests (PFT) and PFT courses during IS treatment were evaluated. RESULTS: EUSTAR contains 3778/11,496 patients with SSc-ILD (33%), with IS in 2681/3,778 (71%). Glucocorticoid (GC) monotherapy was prescribed in 30.6% patients with GC combinations plus cyclophosphamide (CYC) (11.9%), azathioprine (AZA) (9.2%), methotrexate (MTX) (8.7%), or mycophenolate mofetil (MMF) (7.3%). Intensive IS (MMF + GC, CYC or CYC + GC) was started in patients with the worst PFTs and ground glass opacifications on imaging. Patients without IS showed slightly less worsening in forced vital capacity (FVC) when starting with FVC 50-75% or >75%. GC showed negative trends when starting with FVC <50%. Regarding diffusing capacity for carbon monoxide (DLCO), negative DLCO trends were found in patients with MMF. CONCLUSIONS: IS is broadly prescribed in SSc-ILD. Clusters of clinical and functional characteristics guide individualised treatment. Data favour distinguished decision-making, pointing to either watchful waiting and close monitoring in the early stages or start of immunosuppressive treatment in moderately impaired lung function. Advantages of specific IS are difficult to depict due to confounding by indication. Data do not support liberal use of GC in SSc-ILD.
Published: 2018

30. Update of EULAR recommendations for the treatment of systemic sclerosis

Author: UMC Utrecht, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H W, Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller-Ladner, Ulf, Daikeler, Thomas, Lanciano, Elisabetta, Becvár, Radim, Tomcik, Michal, Gindzienska-Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tünde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic-Krstulovic, Daniela, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Morovic-Vergles, Jadranka, Culo, Melanie I., Henes, Jörg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthias F., Klinik, Medizinische, Hasler, Paul, Da Silva, José A Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlos, Midtvedt, Øyvind, Hoffmann-Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre-Sancho, Juan José, Beltran-Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Barešic, Marko, Mayer, Miroslav, üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik-Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma García, Imbert, Bernard, UMC Utrecht, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H W, Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller-Ladner, Ulf, Daikeler, Thomas, Lanciano, Elisabetta, Becvár, Radim, Tomcik, Michal, Gindzienska-Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tünde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic-Krstulovic, Daniela, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Morovic-Vergles, Jadranka, Culo, Melanie I., Henes, Jörg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthias F., Klinik, Medizinische, Hasler, Paul, Da Silva, José A Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlos, Midtvedt, Øyvind, Hoffmann-Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre-Sancho, Juan José, Beltran-Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Barešic, Marko, Mayer, Miroslav, üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik-Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma García, and Imbert, Bernard
Published: 2017

31. Update of EULAR recommendations for the treatment of systemic sclerosis

Author: Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Joerg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Mueller-Ladner, Ulf, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Joerg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, and Mueller-Ladner, Ulf
Abstract: The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatol
Published: 2017

32. Mapping and predicting mortality from systemic sclerosis

Author: Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jerome, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airo, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjak, Laszlo, Tikly, Mohammed, Mueller-Ladner, U. L. F., Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P., Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J., Riccieri, Valeria, Mihai, Carina, Szucs, Gabriella, Walker, Ulrich A., Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Mueller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G., Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Joerg H. W., Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, De langhe, Ellen, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Joerg, Chizzolini, Carlo, von Muhlen, Carlos Alberto, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, De Santis, Maria, Yavuz, Sule, Alberto Sifuentes-Giraldo, Walter, Chatelus, Emmanuel, Stork, Jiri, van Laar, Jacob, Loyo, Esthela, de la Pena Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Jose Alegre-Sancho, Juan, Kowal-Bielecka, Otylia, Rey, Gregoire, Matucci-Cerinic, Marco, Allanore, Yannick, Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jerome, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airo, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjak, Laszlo, Tikly, Mohammed, Mueller-Ladner, U. L. F., Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P., Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J., Riccieri, Valeria, Mihai, Carina, Szucs, Gabriella, Walker, Ulrich A., Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Mueller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G., Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Joerg H. W., Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, De langhe, Ellen, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Joerg, Chizzolini, Carlo, von Muhlen, Carlos Alberto, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, De Santis, Maria, Yavuz, Sule, Alberto Sifuentes-Giraldo, Walter, Chatelus, Emmanuel, Stork, Jiri, van Laar, Jacob, Loyo, Esthela, de la Pena Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Jose Alegre-Sancho, Juan, Kowal-Bielecka, Otylia, Rey, Gregoire, Matucci-Cerinic, Marco, and Allanore, Yannick
Abstract: Objectives To determine the causes of death and risk factors in systemic sclerosis (SSc). Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.
Published: 2017

33. Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population

Author: Hachulla, Eric, Clerson, Pierre, Airò, Paolo, Cuomo, Giovanna, Allanore, Yannick, Caramaschi, Paola, Rosato, Edoardo, Carreira, Patricia E., Riccieri, Valeria, Sarraco, Marta, Denton, Christopher P., Riemekasten, Gabriela, Pozzi, Maria Rosa, Zeni, Silvana, Mihai, Carmen Marina, Ullman, Susanne, Distler, Oliver, Rednic, Simona, Smith, Vanessa, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, Launay, David, Hachulla, Eric, Clerson, Pierre, Airò, Paolo, Cuomo, Giovanna, Allanore, Yannick, Caramaschi, Paola, Rosato, Edoardo, Carreira, Patricia E., Riccieri, Valeria, Sarraco, Marta, Denton, Christopher P., Riemekasten, Gabriela, Pozzi, Maria Rosa, Zeni, Silvana, Mihai, Carmen Marina, Ullman, Susanne, Distler, Oliver, Rednic, Simona, Smith, Vanessa, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, and Launay, David
Abstract: Objective. The aim of this study was to assess the prognostic value of systolic pulmonary artery pressure (sPAP) estimated by echocardiography in the multinational European League Against Rheumatism Scleroderma Trial and Research (EUSTAR) cohort. Methods. Data for patients with echocardiography documented between 1 January 2005 and 31 December 2011 were extracted from the EUSTAR database. Stepwise forward multivariable statistical Cox pulmonary hypertension analysis was used to examine the independent effect on survival of selected variables. Results. Based on our selection criteria, 1476 patients were included in the analysis; 87% of patients were female, with a mean age of 56.3 years (s.d. 13.5) and 31% had diffuse SSc. The mean duration of follow-up was 2.0 years (s.d. 1.2, median 1.9). Taking index sPAP of <30 mmHg as reference, the hazard ratio (HR) for death was 1.67 (95% CI 0.92, 2.96) if the index sPAP was between 30 and 36 mmHg, 2.37 (95% CI 1.14, 4.93) for sPAP between 36 and 40 mmHg, 3.72 (95% CI 1.61, 8.60) for sPAP between 40 and 50 mmHg and 9.75 (95% CI 4.98, 19.09) if sPAP was >50 mmHg. In a multivariable Cox model, sPAP and the diffusing capacity for carbon monoxide (DLCO) were independently associated with the risk of death [HR 1.833 (95% CI 1.035, 3.247) and HR 0.973 (95% CI 0.955, 0.991), respectively]. sPAP was an independent risk factor for death with a HR of 3.02 (95% CI 1.91, 4.78) for sPAP ≥36 mmHg. Conclusion. An estimated sPAP >36 mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization
Published: 2017

34. Update of EULAR recommendations for the treatment of systemic sclerosis

Author: Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Joerg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Mueller-Ladner, Ulf, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Joerg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, and Mueller-Ladner, Ulf
Abstract: The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatol
Published: 2017

35. Mapping and predicting mortality from systemic sclerosis

Author: Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jerome, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airo, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjak, Laszlo, Tikly, Mohammed, Mueller-Ladner, U. L. F., Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P., Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J., Riccieri, Valeria, Mihai, Carina, Szucs, Gabriella, Walker, Ulrich A., Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Mueller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G., Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Joerg H. W., Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, De langhe, Ellen, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Joerg, Chizzolini, Carlo, von Muhlen, Carlos Alberto, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, De Santis, Maria, Yavuz, Sule, Alberto Sifuentes-Giraldo, Walter, Chatelus, Emmanuel, Stork, Jiri, van Laar, Jacob, Loyo, Esthela, de la Pena Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Jose Alegre-Sancho, Juan, Kowal-Bielecka, Otylia, Rey, Gregoire, Matucci-Cerinic, Marco, Allanore, Yannick, Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jerome, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airo, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjak, Laszlo, Tikly, Mohammed, Mueller-Ladner, U. L. F., Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P., Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J., Riccieri, Valeria, Mihai, Carina, Szucs, Gabriella, Walker, Ulrich A., Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Mueller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G., Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Joerg H. W., Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, De langhe, Ellen, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Joerg, Chizzolini, Carlo, von Muhlen, Carlos Alberto, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, De Santis, Maria, Yavuz, Sule, Alberto Sifuentes-Giraldo, Walter, Chatelus, Emmanuel, Stork, Jiri, van Laar, Jacob, Loyo, Esthela, de la Pena Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Jose Alegre-Sancho, Juan, Kowal-Bielecka, Otylia, Rey, Gregoire, Matucci-Cerinic, Marco, and Allanore, Yannick
Abstract: Objectives To determine the causes of death and risk factors in systemic sclerosis (SSc). Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.
Published: 2017

36. Scleroderma and related disorders: 223. Long Term Outcome in a Contemporary Systemic Sclerosis Cohort

Author: Nihtyanova, Svetlana, Ong, Voon, Black, Carol, Denton, Chris, Lutalo, Pamela, Shattles, Warren, Jones, Hugh, Nouri, Reem, Hepburn, Alastair, Chard, Michael, Horwood, Natalie, Lynn, Myo, Duke, Oliver, Kiely, Patrick, Zouita, Louisa, Davies, Ursula, Hughes, Rod, Lloyd, Mark, Nikitorowicz Buniak, Joanna, Shiwen, Xu, Abraham, David, Stratton, Richard, Hügle, Thomas, Schuetz, Philipp, Daikeler, Thomas, Tyndall, Alan, Matucci-Cerinic, Marco, Walker, Ulrich A., van Laar, Jacob M., Pauling, John D., Flower, Victoria, McHugh, Neil, Liu, Shangxi, Leask, Andrew, Aden, Nima, Khan, Korsa, Hoyles, Rachel, Bhagat, Shweta, Drummond, Tom, Goh, Cyndi, Busch, Robert, Hall, Frances, Meyer, Paul, Moinzadeh, Pia, Krieg, Thomas, Hellmich, Martin, Brinckmann, Juergen, Neumann, Elena, Mueller-Ladner, Ulf, Kreuter, Alexander, Dumitresco, Daniel, Rosenkranz, Stefan, Hunzelmann, Nicolas, Binai, Nadine, Huegle, Thomas, van Laar, Jaap, Sonnylal, S., Tam, A., Jones, H., Leask, A., Norman, J., Denton, C., de Crombrugghe, B., Abraham, D., Chighizola, Cecilia B., Luigi Meroni, Pier, Coghlan, Gerry, Newton, Florence, Derrett-Smith, Emma C., Dooley, Audrey, Baliga, Reshma, Hobbs, Adrian, MacAllister, Raymond, Futema, Marta, Pantelidis, Panagiotis, Renzoni, Elizabeth, Schreiber, Benjamin E., Coghlan, Gerry J., Wells, Athol U., Welsh, Ken, Fonseca, Carmen, Ponticos, Markella, Wells, Athol, Guillevin, Loic, Schwierin, Barbara, Rosenberg, Daniel, Silkey, Mariabeth, Parapuram, Sunil, Shi-wen, Xu, Denton, Christopher, Ahmed Abdi, Bahja, Xu, Shiwen, Nihtyanova, Svetlana, Ong, Voon, Black, Carol, Denton, Chris, Lutalo, Pamela, Shattles, Warren, Jones, Hugh, Nouri, Reem, Hepburn, Alastair, Chard, Michael, Horwood, Natalie, Lynn, Myo, Duke, Oliver, Kiely, Patrick, Zouita, Louisa, Davies, Ursula, Hughes, Rod, Lloyd, Mark, Nikitorowicz Buniak, Joanna, Shiwen, Xu, Abraham, David, Stratton, Richard, Hügle, Thomas, Schuetz, Philipp, Daikeler, Thomas, Tyndall, Alan, Matucci-Cerinic, Marco, Walker, Ulrich A., van Laar, Jacob M., Pauling, John D., Flower, Victoria, McHugh, Neil, Liu, Shangxi, Leask, Andrew, Aden, Nima, Khan, Korsa, Hoyles, Rachel, Bhagat, Shweta, Drummond, Tom, Goh, Cyndi, Busch, Robert, Hall, Frances, Meyer, Paul, Moinzadeh, Pia, Krieg, Thomas, Hellmich, Martin, Brinckmann, Juergen, Neumann, Elena, Mueller-Ladner, Ulf, Kreuter, Alexander, Dumitresco, Daniel, Rosenkranz, Stefan, Hunzelmann, Nicolas, Binai, Nadine, Huegle, Thomas, van Laar, Jaap, Sonnylal, S., Tam, A., Jones, H., Leask, A., Norman, J., Denton, C., de Crombrugghe, B., Abraham, D., Chighizola, Cecilia B., Luigi Meroni, Pier, Coghlan, Gerry, Newton, Florence, Derrett-Smith, Emma C., Dooley, Audrey, Baliga, Reshma, Hobbs, Adrian, MacAllister, Raymond, Futema, Marta, Pantelidis, Panagiotis, Renzoni, Elizabeth, Schreiber, Benjamin E., Coghlan, Gerry J., Wells, Athol U., Welsh, Ken, Fonseca, Carmen, Ponticos, Markella, Wells, Athol, Guillevin, Loic, Schwierin, Barbara, Rosenberg, Daniel, Silkey, Mariabeth, Parapuram, Sunil, Shi-wen, Xu, Denton, Christopher, Ahmed Abdi, Bahja, and Xu, Shiwen
Abstract: Background: We have previously compared outcome in two groups of systemic sclerosis (SSc) patients with disease onset a decade apart and we reported data on 5 year survival and cumulative incidence of organ disease in a contemporary SSc cohort. The present study examines longer term outcome in an additional cohort of SSc followed for 10 years. Methods: We have examined patients with disease onset between years 1995 and 1999 allowing for at least 10 years of follow-up in a group that has characteristics representative for the patients we see in contemporary clinical practice. Results: Of the 398 patients included in the study, 252 (63.3%) had limited cutaneous (lc) SSc and 146 (36.7%) had diffuse cutaneous (dc) SSc. The proportion of male patients was higher among the dcSSc group (17.1% v 9.9%, p = 0.037) while the mean age of onset was significantly higher among lcSSc patients (50 ± 13 v 46 ± 13 years ± SD, p = 0.003). During a 10 year follow-up from disease onset, 45% of the dcSSc and 21% of the lcSSc subjects developed clinically significant pulmonary fibrosis, p < 0.001. Among them approximately half reached the endpoint within the first 3 years (23% of dcSSc and 10% of lcSSc) and over three quarters within the first 5 years (34% and 16% respectively). There was a similar incidence of pulmonary hypertension (PH) in the two subsets with a steady rate of increase over time. At 10 years 13% of dcSSc and 15% of lcSSc subjects had developed PH (p=0.558), with the earliest cases observed within the first 2 years of disease. Comparison between subjects who developed PH in the first and second 5 years from disease onset demonstrated no difference in demographic or clinical characteristics, but 5-year survival from PH onset was better among those who developed this complication later in their disease (49% v 24%), with a strong trend towards statistical significance (p = 0.058). Incidence of SSc renal crisis (SRC) was significantly higher among the dcSSc patients (12% v 4%
Published: 2017

37. Nailfold capillary abnormalities in erectile dysfunction of systemic sclerosis: a EUSTAR group analysis

Author: Keck, Andrea D., Foocharoen, Chingching, Rosato, Edoardo, Smith, Vanessa, Allanore, Yannick, Distler, Oliver, Stamenkovic, Bojana, Pereira Da Silva, José Antonio, Hadj Khelifa, Sondess, Denisov, Lev N., Hachulla, Eric, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Airò, Paolo, Caramaschi, Paola, Müller-Ladner, Ulf, Wiland, Piotr, Walker, Ulrich A., Keck, Andrea D., Foocharoen, Chingching, Rosato, Edoardo, Smith, Vanessa, Allanore, Yannick, Distler, Oliver, Stamenkovic, Bojana, Pereira Da Silva, José Antonio, Hadj Khelifa, Sondess, Denisov, Lev N., Hachulla, Eric, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Airò, Paolo, Caramaschi, Paola, Müller-Ladner, Ulf, Wiland, Piotr, and Walker, Ulrich A.
Abstract: Objective. The objective of this study was to analyse an association between nailfold capillary abnormalities and the presence and severity of erectile dysfunction (ED) in men with SSc. Methods. A cross-sectional analysis of the prospective European League Against Rheumatism (EULAR) Scleroderma Trial and Research database was performed. Men with SSc were included if they had undergone nailfold capillaroscopy and simultaneous ED assessment with the 5-item International Index for Erectile Function (IIEF-5). Results. Eighty-six men met the inclusion criteria. Eight men (9.3%) had not had sexual intercourse and could not be assigned an IIEF-5 score. Sixty-nine of the 78 men (88.5%) with an IIEF-5 score had nailfold capillary abnormalities, of whom 54 (78.3%) suffered from ED. Nine men (11.5%) had no nailfold capillary abnormalities, of whom six (66.7%) had ED (P = 0.44). ED was more frequent in older men (P = 0.002) and in men with diffuse disease (P = 0.06). Men with abnormal capillaroscopy had a higher median EULAR disease activity than men without (P = 0.02), a lower diffusing capacity of the lung (P = 0.001) and a higher modified Rodnan skin score (P = 0.04), but mean IIEF-5 scores did not differ [15.7 (s.d. 6.2) vs 15.7 (s.d. 6.3)]. IIEF-5 scores did not differ between men with early (n = 12), active (n = 27) or late (n = 27) patterns (IIEF-5 scores of 17.9, 16.3 and 14.7, respectively). There were no differences in the prevalence of early, active and late capillaroscopy patterns between men with or without ED. Conclusion. Neither the presence or absence of abnormal capillaroscopy findings nor the subdivision into early, active and late patterns is associated with coexistent ED in SSc
Published: 2017

38. Streptococcal hypersensitivity reloaded: severe inflammatory syndrome in Behçet's disease following 23-valent polysaccharide Streptococcus pneumoniae vaccine

Author: Hügle, Thomas, Bircher, Andreas, Walker, Ulrich A., Hügle, Thomas, Bircher, Andreas, and Walker, Ulrich A.
Published: 2017

39. Registries in systemic sclerosis: a worldwide experience

Author: Galluccio, Felice, Walker, Ulrich A., Nihtyanova, Svetlana, Moinzadeh, Pia, Hunzelmann, Nicholas, Krieg, Thomas, Steen, Virginia, Baron, Murray, Sampaio-Barros, Percival, Kayser, Cristiane, Nash, Peter, Denton, Chris P., Tyndall, Alan, Müller-Ladner, Ulf, Matucci-Cerinic, Marco, Galluccio, Felice, Walker, Ulrich A., Nihtyanova, Svetlana, Moinzadeh, Pia, Hunzelmann, Nicholas, Krieg, Thomas, Steen, Virginia, Baron, Murray, Sampaio-Barros, Percival, Kayser, Cristiane, Nash, Peter, Denton, Chris P., Tyndall, Alan, Müller-Ladner, Ulf, and Matucci-Cerinic, Marco
Abstract: SSc is a multisystem disease characterized by an unpredictable course, high mortality and resistance to therapy. The complexity and severity of SSc is a growing burden on the health-care systems. As a result, researchers are seeking new therapeutic strategies for effectively managing these patients. Disease registries are used to support care management efforts for groups of patients with chronic diseases and are meaningful to capture and track key patient information to assist the physicians in managing patients. For these reasons, SSc surveys, research associations and consortiums are pivotal to conduct ongoing research and data collection to enhance disease knowledge and support research projects. Currently, there are several national SSc registries in the UK, Germany, USA, Canada, Brazil and Australia. There is also an international registry established by the European League Against Rheumatism scleroderma trial and research (EUSTAR) called minimal essential data set (MEDS) Online, which collects data from over 8000 patients from 92 centres worldwide, including 21 European centres and 9 centres outside Europe. By collecting, analysing and disseminating data on disease progression and patient responses to long-term disease management strategies, registries help to improve understanding of the disease and keep medical professionals up to date on the latest advances
Published: 2017

40. Do new biologics meet the unmet medical need in rheumatoid arthritis? Safety and efficacy of abatacept following B-cell depletion

Author: Walker, Ulrich A., Courvoisier, Delphine S., Dudler, Jean, Aeberli, Daniel, von Kempis, Johannes, Scherer, Almut, Finckh, Axel, Walker, Ulrich A., Courvoisier, Delphine S., Dudler, Jean, Aeberli, Daniel, von Kempis, Johannes, Scherer, Almut, and Finckh, Axel
Published: 2017

41. Nail fold capillaroscopy differs widely between systemic sclerosis and chronic graft vs host disease of the skin

Author: Aschwanden, Markus, Halter, Jörg P., Walker, Ulrich A., Staub, Daniel, Tichelli, André, Daikeler, Thomas, Jaeger, Kurt A., Tyndall, Alan, Aschwanden, Markus, Halter, Jörg P., Walker, Ulrich A., Staub, Daniel, Tichelli, André, Daikeler, Thomas, Jaeger, Kurt A., and Tyndall, Alan
Published: 2017

42. Skeletal muscle mitochondrial DNA content and aerobic metabolism in patients with antiretroviral therapy-associated lipoatrophy

Author: Sutinen, Jussi, Laaksonen, Marko S., Walker, Ulrich A., Setzer, Bernhard, Kemppainen, Jukka, Nuutila, Pirjo, Yki-Järvinen, Hannele, Sutinen, Jussi, Laaksonen, Marko S., Walker, Ulrich A., Setzer, Bernhard, Kemppainen, Jukka, Nuutila, Pirjo, and Yki-Järvinen, Hannele
Abstract: Objectives To assess whether mitochondrial dysfunction in skeletal muscle characterizes antiretroviral therapy (ART)-associated lipoatrophy (LA). Methods A cross-sectional study comparing HIV-infected, antiretroviral-treated patients with LA (n = 5; LA+) and without LA (n = 5; non-LA) was conducted. Positron emission tomography was used to measure blood flow, oxygen extraction and oxygen consumption in quadriceps femoris muscle during rest and aerobic exercise. Mitochondrial DNA (mtDNA) was quantified by PCR. Body composition was measured by dual-energy X-ray absorptiometry and magnetic resonance imaging. All data are given as means ± SEM. Results Compared with the non-LA group, the LA+ group had significantly less limb fat and more intra-abdominal fat, but similar leg muscle mass. The LA+ group versus the non-LA group had reduced mtDNA content per nucleus in adipose tissue (173 ± 38 versus 328 ± 62; P = 0.067), but not in skeletal muscle (2606 ± 375 versus 2842 ± 309; P = 0.64). Perfusion in resting muscle (34 ± 7 versus 28 ± 6 mL/kg/min in the LA+ group versus the non-LA group; P = 0.5), and the mean absolute (277 ± 30 versus 274 ± 43 mL/kg/min, respectively; P = 0.95) and relative (10.6 ± 2.5- versus 11.9 ± 1.5-fold change, respectively; P = 0.67) increases in perfusion during exercise were similar between the groups. Oxygen consumption at rest (2.2 ± 0.7 versus 2.1 ± 0.3 mL/kg/min in the LA+ group versus the non-LA group; P = 0.9), and the mean absolute (14.6 ± 1.7 versus 24.3 ± 8.8 mL/kg/min, respectively; P = 0.3) and relative (10.3 ± 2.8- versus 11.7 ± 2.4-fold change, respectively; P = 0.73) exercise-induced increases in oxygen consumption were similar between the groups. The oxygen extraction fraction was comparable between the groups, both at rest and during exercise. Plasma lactate concentrations remained unchanged in both groups during exercise. Conclusions HIV-infected patients with ART-associated LA have similar mtDNA content in skeletal muscle and com
Published: 2017

43. Late-onset systemic sclerosis—a systematic survey of the EULAR scleroderma trials and research group database

Author: Hügle, Thomas, Schuetz, Philipp, Daikeler, Thomas, Tyndall, Alan, Matucci-Cerinic, Marco, Walker, Ulrich A., van Laar, Jacob M., Hügle, Thomas, Schuetz, Philipp, Daikeler, Thomas, Tyndall, Alan, Matucci-Cerinic, Marco, Walker, Ulrich A., and van Laar, Jacob M.
Abstract: Objective. The clinical course of SSc depends on subtype, organ involvement and age. Few data are reported on patients suffering from late-onset SSc. Methods. We analysed data from 8554 patients prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) group database. Late-onset SSc was defined as onset of non-RP disease features at or beyond 75 years of age. Disease characteristics, clinical features, disease course and mortality were evaluated. Results. A total of 123 patients with SSc onset at or beyond 75 years of age were identified. Compared with patients <75 years they had more frequently limited than diffuse SSc and a higher prevalence of anti-centromere autoantibodies. Fewer old patients had digital ulcers. The modified Rodnan's skin score, the prevalence of lung fibrosis and renal crisis did not differ significantly between groups. Pulmonary hypertension (PH) measured by echocardiography was more prevalent in the late-onset group, as well as arterial hypertension and diastolic dysfunction. Late-onset SSc remained a positive predictor for PH in multivariate analyses. No significant difference of the two groups in skin score or diffusion capacity was observed during follow-up. Mortality due to SSc was higher in the late-onset group, but the survival time from diagnosis was longer compared with the younger patients. Conclusion. Late-onset SSc shows a distinct clinical presentation and outcome. Patients with late-onset SSc suffer more frequently from the limited subtype and PH, but fewer patients have digital ulcers. PH may in part be determined by underlying cardiovascular disease
Published: 2017

44. Twenty-two points to consider for clinical trials in systemic sclerosis, based on EULAR standards

Author: Khanna, Dinesh, Furst, Daniel E., Allanore, Yannick, Bae, Sangmee, Bodukam, Vijay, Clements, Philip J., Cutolo, Maurizio, Czirjak, Laszlo, Denton, Christopher P., Distler, Oliver, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, Seibold, James R., Singh, Manjit, Tyndall, Alan, Khanna, Dinesh, Furst, Daniel E., Allanore, Yannick, Bae, Sangmee, Bodukam, Vijay, Clements, Philip J., Cutolo, Maurizio, Czirjak, Laszlo, Denton, Christopher P., Distler, Oliver, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, Seibold, James R., Singh, Manjit, and Tyndall, Alan
Abstract: Objective. SSc is clinically and aetiopathogenically heterogeneous. Consensus standards for more uniform trial design and selection of outcome measures are needed. The objective of this study was to develop evidence-based points to consider (PTCs) for future clinical trials in SSc. Methods. Thirteen international SSc experts experienced in SSc clinical trial design were invited to participate. One researcher with experience in systematic literature review and three trainees were also included. A systematic review using PubMed and the Cochrane Central Register of Controlled Trials was conducted and PTCs when designing clinical trials in SSc were developed. As part of that development we conducted an Internet-based Delphi exercise regarding the main points to be made in the consensus statement. Consensus was defined as achieving a median score of ≥7 of 9. Results. By consensus, the experts decided to develop PTCs for each individual organ system. The current document provides a unifying outline on PTCs regarding general trial design, inclusion/exclusion criteria and analysis. Consensus was achieved regarding all the main points of the PTCs. Conclusion. Using European League Against Rheumatism suggestions for PTCs, a general outline for PTCs for controlled clinical trials in SSc was developed. Specific outlines for individual organ systems are to be published separately. This general outline should lead to more uniform and higher-quality trials and clearly delineate areas where further research is needed
Published: 2017

45. Systemic sclerosis without antinuclear antibodies or Raynaud's phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database

Author: Schneeberger, Daniel, Tyndall, Alan, Kay, Jonathan, Søndergaard, Klaus H., Carreira, Patricia E., Morgiel, Ewa, Deuschle, Katrin, Derk, Chris T., Widuchowska, Malgorzata, Walker, Ulrich A., Schneeberger, Daniel, Tyndall, Alan, Kay, Jonathan, Søndergaard, Klaus H., Carreira, Patricia E., Morgiel, Ewa, Deuschle, Katrin, Derk, Chris T., Widuchowska, Malgorzata, and Walker, Ulrich A.
Abstract: Objective. To assess patients with SSc who present without circulating ANAs or RP. Methods. Five thousand three hundred and ninety patients who fulfilled the ACR criteria for SSc and were enrolled in the EULAR Scleroderma Trials and Research (EUSTAR) database were screened for the absence of both RP and circulating ANA. To differentiate SSc from its mimics, additional information was gathered using a standardized questionnaire. Results. Five thousand three hundred and seventy-eight (99.8%) of the 5390 SSc patients in the EUSTAR database had either detectable ANA or a history of RP. Twelve (0.2%) patients lacked both circulating ANA and RP. Details of the medical history could be obtained for seven patients. Three cases were compatible with ANA-negative and RP-negative SSc and were not typical of any known SSc mimic. Four patients had a malignancy: two had breast cancer, one had multiple myeloma with possible scleromyxoedema and one had bladder carcinoma. There was no temporal relationship between the onset of skin fibrosis and that of the tumour. Although no patient with confirmed nephrogenic systemic fibrosis was identified among the cases of ANA-negative and RP-negative SSc, the presentation of one patient could be compatible with that of nephrogenic systemic fibrosis other than for the absence of chronic kidney disease or of known prior gadolinium exposure. Conclusion. We have identified a very small subgroup of SSc patients who lack both circulating ANA and RP, none of whom fulfils the diagnostic criteria for any known SSc mimic. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients
Published: 2017

46. Disease activity dynamics in rheumatoid arthritis: patients’ self-assessment of disease activity via WebApp

Author: Walker, Ulrich A, Mueller, Ruediger B, Jaeger, Veronika K, Theiler, Robert, Forster, Adrian, Dufner, Patrick, Ganz, Fabiana, Kyburz, Diego, Walker, Ulrich A, Mueller, Ruediger B, Jaeger, Veronika K, Theiler, Robert, Forster, Adrian, Dufner, Patrick, Ganz, Fabiana, and Kyburz, Diego
Published: 2017

47. Update of EULAR recommendations for the treatment of systemic sclerosis.

Author: UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, Del Galdo, Francesco, Denton, Christopher P, Distler, Jörg H W, Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E, Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L, van den Hoogen, Frank, van Laar, Jacob M, Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A, Zulian, Francesco, Müller-Ladner, Ulf, EUSTAR Coauthors, Vanthuyne, Marie, UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, Del Galdo, Francesco, Denton, Christopher P, Distler, Jörg H W, Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E, Guiducci, Serena, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L, van den Hoogen, Frank, van Laar, Jacob M, Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A, Zulian, Francesco, Müller-Ladner, Ulf, EUSTAR Coauthors, and Vanthuyne, Marie
Abstract: The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research (EUSTAR) group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis, and gastrointestinal involvement. Compared with the 2009 recommendations, the 2015 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressing SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help
Published: 2016

48. Functional Disability and Its Predictors in Systemic Sclerosis: A Study from the Desscipher Project within the European Scleroderma Trials and Research Group

Author: Jaeger, Veronika K., Czirjak, Lazlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Distler, Oliver, Maurer, Britta, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriela, Siegert, Elise, Huscher, Dorte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Garay-Toth, Beata, Ananieva, Lidiya P., Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Vacca, Alessandra, Schmeiser, Tim, Rednic, Simona, Riccieri, Valeria, Krummel-Lorenz, Brigitte, Gabrielli, Armando, De la Pena, Paloma Garcia, Ancuta, Codrina, Muller-Ladner, Ulf, Walker, Ulrich A., Jaeger, Veronika K., Czirjak, Lazlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Distler, Oliver, Maurer, Britta, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriela, Siegert, Elise, Huscher, Dorte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Garay-Toth, Beata, Ananieva, Lidiya P., Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Vacca, Alessandra, Schmeiser, Tim, Rednic, Simona, Riccieri, Valeria, Krummel-Lorenz, Brigitte, Gabrielli, Armando, De la Pena, Paloma Garcia, Ancuta, Codrina, Muller-Ladner, Ulf, and Walker, Ulrich A.
Published: 2016

49. Functional Disability and Its Predictors in Systemic Sclerosis: A Study from the Desscipher Project within the European Scleroderma Trials and Research Group

Author: Jaeger, Veronika K., Czirjak, Lazlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Distler, Oliver, Maurer, Britta, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriela, Siegert, Elise, Huscher, Dorte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Garay-Toth, Beata, Ananieva, Lidiya P., Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Vacca, Alessandra, Schmeiser, Tim, Rednic, Simona, Riccieri, Valeria, Krummel-Lorenz, Brigitte, Gabrielli, Armando, De la Pena, Paloma Garcia, Ancuta, Codrina, Muller-Ladner, Ulf, Walker, Ulrich A., Jaeger, Veronika K., Czirjak, Lazlo, Lorand, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Distler, Oliver, Maurer, Britta, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriela, Siegert, Elise, Huscher, Dorte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H., Garay-Toth, Beata, Ananieva, Lidiya P., Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Vacca, Alessandra, Schmeiser, Tim, Rednic, Simona, Riccieri, Valeria, Krummel-Lorenz, Brigitte, Gabrielli, Armando, De la Pena, Paloma Garcia, Ancuta, Codrina, Muller-Ladner, Ulf, and Walker, Ulrich A.
Published: 2016

50. Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study

Author: Jaeger, Veronika K, Wirz, Elina G, Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airò, Paolo, Carreira, Patricia E, Balbir Gurman, Alexandra, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Müller-Ladner, Ulf, Distler, Jörg H W, Li, Mangtao, Walker, Ulrich A, EUSTAR co-authors, Jaeger, Veronika K, Wirz, Elina G, Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airò, Paolo, Carreira, Patricia E, Balbir Gurman, Alexandra, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Müller-Ladner, Ulf, Distler, Jörg H W, Li, Mangtao, Walker, Ulrich A, and EUSTAR co-authors
Abstract: OBJECTIVE Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. METHODS In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. RESULTS Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide<80% predicted (65%), DU (34%), cardiac involvement (32%), FVC<80% predicted (31%), increased PAPsys>40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. CONCLUSION In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to 'widen' the still very narrow 'window of opportunity'. They may also enable physicians to counsel and manage patients presentin
Published: 2016

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