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1. 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 HW, 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, EUSTAR Collaborators, Guiducci, S., Walker, U., Kyburz, D., Lapadula, G., Iannone, F., Maurer, B., Jordan, S., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Siegert, E., Rednic, S., Nicoara, I., Kahan, A., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Stork, J., Inanc, M., Carreira, P.E., Novak, S., Czirják, L., Varju, C., Chizzolini, C., Kucharz, E.J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Wu, C., Marjanovic, Z., Faivre, H., Hij, D., Dhamadi, R., Airò, P., Hesselstrand, R., Wollheim, F., Wuttge, D.M., Andréasson, K., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Trotta, F., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Mauriziano, O., Maria Bambara, L., Caramaschi, P., Morovic-Vergles, J., Black, C., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Burkhardt, H., Himsel, A., Bajocchi, G., Maria Nuova, A.S., João Salvador, M., Pereira Da Silva, J.A., Stamenkovic, B., Stankovic, A., Francesco Selmi, C., De Santis, M., Marasini, B., Tikly, M., Ananieva, L.P., Denisov, L.N., Müller-Ladner, U., Frerix, M., Tarner, I., Scorza, R., Puppo, F., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szücs, G., Szamosi, S., Zea Mendoza, A., de la Puente, C., Sifuentes Giraldo, W.A., Midtvedt, Ø., Reiseter, S., Garen, T., Valesini, G., Riccieri, V., Maria Ionescu, R., Opris, D., Groseanu, L., Wigley, F.M., Sfrent Cornateanu, R., Ionitescu, R., Maria Gherghe, A., Soare, A., Gorga, M., Bojinca, M., Mihai, C., Milicescu, M., Sunderkötter, C., Kuhn, A., Sandorfi, N., Schett, G., Distler, J.H., Beyer, C., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Paolo Cantatore, F., Corrado, A., Ullman, S., Iversen, L., Alberto von Mühlen, C., Marilu Bohn, J., Scussel Lonzetti, L., Rosa Pozzi, M., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Houssiau, F.A., Jose Alegre-Sancho, J., Krummel-Lorenz, B., Saar, P., Aringer, M., Günther, C., Westhovens, R., de Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Cezar Radominski, S., de Souza Müller, C., Azevedo, V.F., Jimenez, S., Busquets, J., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Popa, S., Zenone, T., Pileckyte, M., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Sampaio-Barros, P.D., Yoshinari, N.H., Marangoni, R.G., Martin, P., Fuocco, L., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Abdel Atty Mohamed, W.A., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C.M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Chirieac, R., Ancuta, C., Furst, D.E., Villiger, P., Adler, S., van Laar, J., Kayser, C., Eduardo C, A.L., Fathi, N., Hassanien, M., de la Peña Lefebvre, P.G., Rodriguez Rubio, S., Valero Exposito, M., Sibilia, J., Chatelus, E., Gottenberg, J.E., Chifflot, H., Litinsky, I., Emery, P., Buch, M., Del Galdo, F., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Ann Saketkoo, L., Lasky, J.A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Luca Brucato, A., Lupi, E., Rosner, I., Rozenbaum, M., Slobodin, G., Boulman, N., Rimar, D., Couto, M., Spertini, F., Ribi, C., Buss, G., Kahl, S., Hsu, V.M., Chen, F., McCloskey, D., Malveaux, H., Louis Pasquali, J., Martin, T., Gorse, A., Guffroy, A., Poindron, V., and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Databases, Factual, systemic sclerosis, SUBSETS, Disease, Severity of Illness Index, Scleroderma, DISEASE, 0302 clinical medicine, Medicine and Health Sciences, Immunology and Allergy, Cluster Analysis, CRITERIA, Prospective Studies, Prospective cohort study, skin and connective tissue diseases, integumentary system, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Adult, Aged, Autoantibodies/blood, Europe/epidemiology, Female, Humans, Middle Aged, Phenotype, Prognosis, Scleroderma, Diffuse/blood, Scleroderma, Diffuse/epidemiology, Scleroderma, Diffuse/pathology, Scleroderma, Limited/blood, Scleroderma, Limited/epidemiology, Scleroderma, Limited/pathology, Scleroderma, Systemic/blood, Scleroderma, Systemic/epidemiology, Scleroderma, Systemic/pathology, Connective tissue disease, ddc, Europe, MANIFESTATIONS, Cohort, Life Sciences & Biomedicine, medicine.medical_specialty, Immunology, PROFILE, CLASSIFICATION, 03 medical and health sciences, Rheumatology, Scleroderma, Limited, Internal medicine, Severity of illness, medicine, Autoantibodies, 030203 arthritis & rheumatology, Science & Technology, Scleroderma, Systemic, business.industry, Autoantibody, Systemic sclerosis (SSc), medicine.disease, 030104 developmental biology, Scleroderma, Diffuse, business
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. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

2. 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, Cometi, L, Czirják, László, Denton, Christopher P, Distler, Oliver, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A, Allanore, Yannick, Müller-Ladner, Ulf, Del Galdo, Francesco, Matucci-Cerinic, Marco, EUSTAR co-workers, University of Zurich, Blagojevic, Jelena, Blagojevic, J., Bellando-Randone, S., Abignano, G., Avouac, J., Cometi, L., Czirják, L., Denton, C. P., Distler, O., Frerix, M., Guiducci, S., Huscher, D., Jaeger, V. K., Lóránd, V., Maurer, B., Nihtyanova, S., Riemekasten, G., Siegert, E., Tarner, I. H., Vettori, S., Walker, U. A., Allanore, Y., Müller-Ladner, U., Del Galdo, F., and Matucci-Cerinic, M.

Subjects
0301 basic medicine, Male, lcsh:Diseases of the musculoskeletal system, Observational Trial, 2745 Rheumatology, Digital ulcer, Categorisation, Scleroderma, Systemic sclerosi, 0302 clinical medicine, Surveys and Questionnaires, Immunology and Allergy, Prospective Studies, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, Calcium Channel Blockers, Classification, 3. Good health, Clinical Practice, Categorization, 2723 Immunology and Allergy, Systemic sclerosis, Drug Therapy, Combination, Female, Research Article, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, Sildenafil Citrate, Fingers, 03 medical and health sciences, Rheumatology, Skin Ulcer, medicine, Humans, In patient, European Union, Iloprost, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Bosentan, Essential item, medicine.disease, 030104 developmental biology, Essential items, Physical therapy, Observational study, lcsh:RC925-935, business
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 while further implementation of DU classification and categorisation is warranted.Trial registration: Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013).

Published
2018

3. Atherosclerotic plaques occur in absence of intima-media thickening in both systemic sclerosis and systemic lupus erythematosus : a duplexsonography study of carotid and femoral arteries and follow-up for cardiovascular events

Author

Frerix, Marc, Stegbauer, Johannes, Kreuter, Alexander, Weiner, Stefan, and Department of Internal Medicine and Rheumatology

Subjects
Rheumatology, Immunology, Immunology and Allergy, ddc:610, skin and connective tissue diseases, Medical sciences Medicine
Abstract

INTRODUCTION: The objective of this cross-sectional and retrospective cohort study was (1) to determine the usefulness of intima-media thickness (IMT) in contrast to plaque assessment, (2) to examine the value of additive femoral artery sonography and (3) to identify potential risk factors for atherosclerosis and incident cardiovascular events in systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) patients. METHODS: In this study, 90 SSc and 100 SLE patients were examined by duplexsonography. IMT was measured in common carotid and common femoral arteries, plaques were assessed in common, internal and external carotid and common, proximal superficial and deep femoral arteries. Different definitions of pathological IMT (pIMT) were compared with the presence of plaque. Results were evaluated in relation to traditional and non-traditional risk factors for baseline atherosclerosis (logistic regression) and their predictive value for cardiovascular events during follow-up (cox regression). RESULTS: Definite atherosclerosis occurred frequently without signs of subclinical atherosclerosis in both diseases: pIMT >0.9mm was present in only 17/59 (28.9%) SSc and 13/49 (26.5%) SLE patients with already present atherosclerotic plaques. Using age-adjusted pIMT definitions, this rate was even lower (5.1-10.3% in SSc, 14.3-26.5% in SLE). Plaques were located only at the carotid or only at the femoral arteries in 26 (13.7%) and 24 (12.6%) patients, respectively. Age and nicotine pack-years were independently associated with atherosclerotic plaques in SLE and SSc patients, as well as the cumulative prednisolone dose in SSc subgroup, and ssDNA positive SLE patients had a lower risk for atherosclerotic plaque. During follow-up (available for 129/190 (67.9%) patients, 650 person-years), cardiovascular events occurred more often in patients with coronary heart disease (adjusted-hazards ratio (HR) 10.19, 95% confidence interval (CI) 3.04 to 34.17, P

Published
2014

4. Intravenous sildenafil acutely improves hemodynamic response to exercise in patients with connective tissue disease.

Author

Rieth, Andreas J., Richter, Manuel J., Berkowitsch, Alexander, Frerix, Marc, Tarner, Ingo H., Mitrovic, Veselin, and Hamm, Christian W.

Subjects
SILDENAFIL, CYCLIC nucleotide phosphodiesterase inhibitors, EXERCISE, CONNECTIVE tissue diseases, MUSCULOSKELETAL system diseases
Abstract

Background: Hemodynamic assessment during exercise may unmask an impaired functional reserve of the right ventricle and the pulmonary vasculature in patients with connective tissue disease. We assessed the effect of intravenous sildenafil on the hemodynamic response to exercise in patients with connective tissue disease. Methods: In this proof-of-concept study, patients with connective tissue disease and mean pulmonary arterial pressure (mPAP) >20 mm Hg were subjected to a supine exercise hemodynamic evaluation before and after administration of intravenous sildenafil 10 mg. Results: Ten patients (four with moderately elevated mPAP 21–24 mm Hg; six with mPAP >25 mm Hg) underwent hemodynamic assessment. All of them showed markedly abnormal exercise hemodynamics. Intravenous sildenafil was well tolerated and had significant hemodynamic effects at rest and during exercise, although without pulmonary selectivity. Sildenafil reduced median total pulmonary resistance during exercise from 6.22 (IQR 4.61–8.54) to 5.24 (3.95–6.96) mm Hg·min·L-1 (p = 0.005) and increased median pulmonary arterial capacitance during exercise from 1.59 (0.93–2.28) to 1.74 (1.12–2.69) mL/mm Hg (p = 0.005). Conclusions: In patients with connective tissue disease who have an abnormal hemodynamic response to exercise, intravenous sildenafil improved adaption of the right ventricular-pulmonary vascular unit to exercise independent of resting mPAP. The impact of acute pharmacological interventions on exercise hemodynamics in patients with pulmonary vascular disease warrants further investigation. Trial registration: Clinicaltrials.gov . [ABSTRACT FROM AUTHOR]

Published
2018
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5. 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, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, and Hunzelmann, Nicolas

Subjects
ACE inhibitors, FLUOXETINE, GASTROINTESTINAL disease treatment, THERAPEUTIC use of glucocorticoids, KIDNEY disease treatments, LUNG disease treatment, PULMONARY hypertension treatment, ULCER treatment, HETEROCYCLIC compounds, SEROTONIN uptake inhibitors, PHOSPHODIESTERASE inhibitors, PROSTAGLANDINS I, DELPHI method, ENDOTHELINS, FINGERS, GASTROINTESTINAL diseases, HEMATOPOIETIC stem cell transplantation, KIDNEY diseases, LUNG diseases, PULMONARY hypertension, RAYNAUD'S disease, RHEUMATOLOGY, SYSTEMIC scleroderma, ULCERS, CHEMICAL inhibitors, DISEASE complications, THERAPEUTICS
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 rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc. [ABSTRACT FROM AUTHOR]

Published
2017
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6. Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group

Author

Christopher P. Denton, Francesco DelGaldo, László Czirják, Gabriele Valentini, Elise Siegert, Sabine Adler, Peter M. Villiger, Marco Matucci-Cerinic, Dörte Huscher, Ulrich A Walker, Yannick Allanore, Marc Frerix, Ulf Mueller-Ladner, Ingo-Helmut Tarner, Oliver Distler, Gabriela Riemekasten, Adler, Sabine, Huscher, Dã¶rte, Siegert, Elise, Allanore, Yannick, Czirjã¡k, Lã¡szlã³, Delgaldo, Francesco, Denton, Christopher P., Distler, Oliver, Frerix, Marc, Matucci-Cerinic, Marco, Mueller-Ladner, Ulf, Tarner, Ingo-Helmut, Valentini, Gabriele, Walker, Ulrich A., Villiger, Peter M., Riemekasten, Gabriela, and University of Zurich

Subjects
Male, Vital capacity, lcsh:Diseases of the musculoskeletal system, 2745 Rheumatology, Azathioprine, Pulmonary function testing, Systemic sclerosi, 0302 clinical medicine, DLCO, Diffusing capacity, Immunology and Allergy, Precision Medicine, skin and connective tissue diseases, Lung, Immunosuppressant, Interstitial lung disease, 10051 Rheumatology Clinic and Institute of Physical Medicine, respiratory system, Middle Aged, 3. Good health, Respiratory Function Tests, 2723 Immunology and Allergy, Systemic sclerosis, Drug Therapy, Combination, Female, Immunosuppressive Agents, medicine.drug, Research Article, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, Internal medicine, medicine, Humans, Cyclophosphamide, Aged, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Follow up, Mycophenolic Acid, medicine.disease, Lung function, respiratory tract diseases, Immunosuppressants, Methotrexate, 030228 respiratory system, lcsh:RC925-935, business, Lung Diseases, Interstitial, Rheumatism
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

Published
2017

7. The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: Derivation and validation of a preliminarily revised EUSTAR activity index

Author

Ulrich A Walker, Oliver Distler, Suzana Jordan, Gabriele Valentini, Otylia Kowal-Bielecka, Serena Vettori, Gabriela Riemekasten, Elise Siegert, Murray Baron, Voon H Ong, Michele Iudici, Jérôme Avouac, Janet E. Pope, Yannick Allanore, Veronika K. Jaeger, Marc Frerix, Eric Hachulla, Ariane L. Herrick, László Czirják, Ulf Müller-Ladner, Tünde Minier, Patricia Carreira, Christopher P. Denton, Valentini, Gabriele, Iudici, Michele, Walker, Ulrich A., Jaeger, Veronika K., Baron, Murray, Carreira, Patricia, Czirják, László, Denton, Christopher P., Distler, Oliver, Hachulla, Eric, Herrick, Ariane L., Kowal Bielecka, Otylia, Pope, Janet, Müller Ladner, Ulf, Riemekasten, Gabriela, Avouac, Jerome, Frerix, Marc, Jordan, Suzana, Minier, Tünde, Siegert, Elise, Ong, Voon H., Vettori, Serena, Allanore, Yannick, and University of Zurich

Subjects
0301 basic medicine, Male, 2745 Rheumatology, Activity index, Severity of Illness Index, Scleroderma, 0302 clinical medicine, DLCO, Diffusing capacity, Medicine and Health Sciences, Medicine, Immunology and Allergy, Aged, 80 and over, Medicine (all), 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Europe, 2723 Immunology and Allergy, Female, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, Sensitivity and Specificity, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Young Adult, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Linear regression, Humans, Derivation, Disease Activity, Aged, Systemic Sclerosi, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, Biochemistry, Genetics and Molecular Biology (all), business.industry, Reproducibility of Results, Gold standard (test), medicine.disease, 030104 developmental biology, ROC Curve, Physical therapy, Observational study, business, Follow-Up Studies
Abstract

BackgroundValidity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised.MethodsThree investigators assigned an activity score on a 0–10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate–multivariate linear regression analyses were used to define variables predicting the ‘gold standard’, their weight and derive an activity index. The cut-off point of the index best separating active/very active from inactive/moderately active disease was identified by a receiver-operating curve analysis. The index was validated on a second set of 60 charts assessed by three different investigators on a 0–10 scale and defined as inactive/moderately active or active/very active by other two investigators. One hundred and twenty-three were investigated for changes over time in the index and their relationships with those in the summed Medsger severity score (MSS).ResultsA weighted 10-point activity index was identified and validated: Δ-skin=1.5 (Δ=patient assessed worsening during the previous month), modified Rodnan skin score (mRss) >18=1.5, digital ulcers=1.5, tendon friction rubs=2.25, C-reactive protein >1 mg/dL=2.25 and diffusing capacity of the lung for CO (DLCO) % predicted ConclusionsA preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies.

Published
2017

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

Author

Ulf Müller-Ladner, Beat Fankhauser, Valeria Riccieri, Marco Matucci-Cerinic, Christopher P. Denton, Jörg Umbricht, Brigitte Krummel-Lorenz, Franco Cozzi, László Czirják, Jérôme Avouac, Ulrich A Walker, Alessandra Vacca, Armando Gabrielli, Serena Vettori, Yannick Allanore, Marc Frerix, Francesco Del Galdo, Giuseppina Abignano, Paloma García de la Peña Lefebvre, Dörte Huscher, Veronika Lóránd, Carina Mihai, Ingo H. Tarner, Britta Maurer, Gabriele Valentini, Elise Siegert, T. Schmeiser, Nicolas Hunzelmann, Simona Rednic, G. Riemekasten, Beata Garay Toth, Serena Guiducci, Svetlana I. Nihtyanova, Codrina Ancuta, Sule Yavuz, Oliver Distler, Vanessa Smith, Anastasia Zakharova, Veronika K. Jaeger, Duska Martinovic, Jaeger, Veronika K, Distler, Oliver, Maurer, Britta, Czirják, Laszlo, Lóránd, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Dörte, Matucci Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H, Garay Toth, Beata, Fankhauser, Beat, Umbricht, Jörg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicola, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, García de la Peña Lefebvre, Paloma, Gabrielli, Armando, Krummel Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Müller Ladner, Ulf, and Walker, Ulrich A.

Subjects
medicine.medical_specialty, functional disability, systemic sclerosis, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Quality of life, Internal medicine, Severity of illness, medicine, Functional disability, Predictors of disability, Scleroderma health assessment questionnaire, Systemic sclerosis, Europe, Gastrointestinal Diseases, Humans, Hypertension, Pulmonary, Longitudinal Studies, Muscle Weakness, Pain Measurement, Prospective Studies, Regression Analysis, Risk Factors, Scleroderma, Systemic, Severity of Illness Index, Skin Ulcer, Activities of Daily Living, Disability Evaluation, Quality of Life, Sickness Impact Profile, Pharmacology (medical), 030212 general & internal medicine, Prospective cohort study, 030203 arthritis & rheumatology, business.industry, predictors of disability, scleroderma health assessment questionnaire, Muscle weakness, sistemska skleroza, funkcionalna invalidnost, zdravstveni upitnik, prediktori invaliditeta, Skin ulcer, medicine.disease, Pulmonary hypertension, Rheumatoid arthritis, Cohort, Physical therapy, Functional disability, systemic sclerosis, medicine.symptom, business
Abstract

Objectives: 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. Methods: 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. Results: 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 ⩽ SHAQ < 1), 34% in the moderate to severe disability category (1 ⩽ SHAQ < 2) and 7% in the severe to very severe disability category (2 ⩽ SHAQ ⩽ 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 vs 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). Conclusion: 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
2017

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