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1. Enthesopathy as early manifestation in psoriatic arthritis

Author

Eugeniu Russu, Liliana Groppa, Lia Chislari, Victor Cazac, and Svetlana Agachi

Subjects
psoriatic arthritis, enthesopathy, ultrasound, Medicine
Abstract

Background: Despite the progress made in the study of psoriasis and psoriatic arthritis, their early diagnosis and treatment for practicing physicians continue to be a difficult problem. Material and methods: 100 people were examined, including 70 patients with psoriatic arthritis aged between 18 and 60 years (23 men and 47 women), admitted to the rheumatology and arthrology departments of the Timofei Mosneaga Republican Clinical Hospital 2019-2022 (Favorable opinion of the Committee for Research Ethics, No 21 of 21.12.2019). The control group included 30 people with rheumatoid arthritis. Results: Ultrasound signs of damage to the joint structures were detected, such as synovitis (p=0.26), cartilage changes (p=0.433), enthesopathy (p=0.980) and tenosynovitis, statistically significant differences (p=0.800). Magnetic resonance imaging determined that fluid was the predominant symptom in frequency (n=13, 92.86%), including in the small joints of the hands (n=1, 100%) and feet (n=2, 100%). Conclusions: In large joints, the proliferation of the synovial membrane was detected in 51.67% of the joints and had predominantly high echogenicity. At small joints, synovial proliferation with predominantly low echogenicity occurred only in 6.1% of the joints.

Published
2022
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2. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort

Author

Pavel I Novikov, Thierry Martin, Eric Hachulla, Vanessa Smith, Maurizio Cutolo, Yannick Allanore, Camillo Ribi, Oliver Distler, Britta Maurer, Paolo Airo, Cosimo Bruni, Veronica Codullo, Luc Mouthon, Marie Vanthuyne, Elisabetta Zanatta, Simona Rednic, Ulf Müller-Ladner, Nemanja Damjanov, Anna-Maria Hoffmann-Vold, Michele Iudici, Suzana Jordan, Jean Sibilia, Mengtao Li, Roger Hesselstrand, Elise Siegert, László Czirják, Armando Gabrielli, Dominique Farge Bancel, Yolanda Braun-Moscovici, Nicolas Hunzelmann, Raffaele Pellerito, Jörg Henes, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Merete Engelhart, Valeria Riccieri, Cord Sunderkötter, Francesca Ingegnoli, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Piotr Wiland, Juan Jose Alegre-Sancho, Brigitte Krummel-Lorenz, Ellen De Langhe, Branimir Anic, Carolina de Souza Müller, Simon Stebbings, Alessandra Vacca, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Edoardo Rosato, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Tim Schmeiser, Piercarlo Sarzi Puttini, Fabio Cacciapaglia, Marie-Elise Truchetet, Muriel Elhai, Juan José Alegre Sancho, Ivan Castellví, Serena Vettori, Vivien M Hsu, Masataka Kuwana, Sule Yavuz, Radim Becvar, Patricia E Carreira, Eugene J Kucharz, Bernard Coleiro, Paul Hasler, Carlos de la Puente, Svetlana Agachi, Thierry Zenone, Francesco Del Galdo, Doron Rimar, Alessandro Giollo, Lidia P Ananieva, Ina Kötter, Mislav Radic, Dominik Majewski, Kristine Herrmann, Goda Seskute, Lorinda S Chung, Laura Belloli, Ulrike Held, FJ López-Longo, Joerg H W Distler, Maria João Salvador, Simon Kuster, Klaus Steigmiller, Ana-Maria Gheorghiu, Katarzyna Romanowska-Prochnicka, Yuichiro Shirai, Chris Denton, Ruxandra M Ionescu, Stefan Heitmann Vera Ortiz-Santamaria, Walid Ahmed Abdel Atty Mohamed, Paloma Garcíadela Peña Lefebvre, and Vera Bernardino

Subjects
Medicine
Abstract

Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis (SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database.Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12±3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12±3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months.Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference −1.0, 95% CI −3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (−6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles.Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population.

Published
2022
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3. Sclerodermia sistemică la bărbat. Caz clinic.

Author

Rodica Surchiceanu, Liliana Groppa, Lucia Dutca, Svetlana Agachi, and Larisa Rotaru

Subjects
sclerodermie sistemică, bărbat, țesut conjunctiv, Medicine (General), R5-920, Internal medicine, RC31-1245, Other systems of medicine, RZ201-999, Public aspects of medicine, RA1-1270
Abstract

Sclerodermia sistemică (Ssc) este o maladie generalizată a țesutului conjunctiv caracterizată prin dezvoltarea manifestărilor patologice cutanate, ale aparatului locomotor a organelor interne (cordului, pulmonilor, rinichilor, tractului gastrointestinal) și a dereglărilor vasospastice difuze cauzate de afectarea țesutului conjunctiv cu predominarea fibrozei și de alterarea vasculară de tipul microangiopatiei obliterante. Aproximativ 15% până la 25% dintre persoanele cu trăsături de sclerodermie sistemică prezintă, de asemenea, simptome ale unei alte afecțiuni care afectează țesutul conjunctiv, cum ar fi polimiozita, dermatomiozita, artrita reumatoidă, sindromul Sjögren sau lupusul eritematos sistemic. Prevalența sclerodermiei sistemice este estimată a varia între 50 și 300 de cazuri la 1 milion de persoane. Din motive necunoscute, femeile au de patru ori mai multe șanse să dezvolte afecțiunea decât bărbații. Raportăm cazul clinic al unui pacient de 45 de ani cu semne clinice și paraclinice de SSc.

Published
2020

4. Particularitățile evolutive și de tratament în glomerulonefritele cronice.

Author

Alina Struță, Liliana Groppa, Svetlana Agachi, Dorian Sasu, Tatiana Răzlog, and Larisa Rotaru

Subjects
glomerulonefrite cronice, evoluţie, tratament, prognostic, Medicine (General), R5-920, Internal medicine, RC31-1245, Other systems of medicine, RZ201-999, Public aspects of medicine, RA1-1270
Abstract

Glomerulonefrita cronică se dezvoltă treptat, poate fi depistată ocazional în absența simptoamelor [19]. Varianta cronică a bolii nu are o cauză certă. Glomerulonefritele cronice sunt cauza de la 35% până la 55% din insuficiență renală în stadiu terminal, în funcție de țări sau regiuni [20, 22]. Scopul acestei lucrări - stabilirea și evaluarea prognosticului glomerulonefritelor cronice în dependență de tratamentul aplicat la pacienții cu glomerulonefrită cronică. A fost stabilit - în dependență de sindromul clinic principal prezent: 29 (48,3%) pacienți – au fost cu forma mixta, 16 (26,6%) pacienți – cu sindromul nefritic, 11 (18,3%) pacienți – cu sindromul nefrotic, 3 (5%) pacient – cu forma hematurica și 1 (1,6%) pacient – cu sindromul hipertensiv. După tratamentul aplicat: 57 (95%) din toți pacienți primesc terapie antihipertensivă; 52 (86,6%) pacienți primesc diuretice; 49 (81,6%) pacienți primesc statine; 31 (51,6%) pacienți administrează glucocorticosteroizi; 29 (48,3%) pacienți primesc citostatice; 20 (33,3%) sunt cu terapie antianemica.

Published
2020

5. ONSET OF CLINICAL MANIFESTATIONS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Author

Mirela Curchi, Elena Deseatnicova, Svetlana Agachi, Larisa Rotaru, and Liliana Groppa

Subjects
Systemic lupus erythematosus, clinical features, Medicine, Surgery, RD1-811
Abstract

Objectives. Systemic lupus erythematosus is a chronic autoimmune disease that can affect any organ or system. It develops on the background of genetically imperfect immunity, associated with hyperproduction of anti-nuclear antibodies. It is characterized by a variety of clinical signs. The aim of the study was to determine specific clinical features in patients with systemic lupus erythematosus. Material and methods. A total of 64 patients with systemic lupus erythematosus arthritis were examined, which were consecutively admitted in the Rheumatology Department of the Clinical Republican Hospital ”Timofei Moșneaga”, Chișinău, Republic of Moldova, in 2019. To determine the clinical features of patients, a questionnaire was completed, according to the diagnostic criteria, risc factor, etc. The data obtained was statistically analyzed. Results. Of all examined patients, 52 (81.4%) were women and 12 (18.6%) were men. The mean age of the examined patients was 48.3 ± 8.7 years, the duration of the disease was, in average, 12.15 ± 9.32 years. In the examined group, 98% had musculoskeletal manifestations and, at the same time, 85% showed mucocutaneous manifestations. The most common sign was alopecia at 89.06%, and malar rush at 60.9% of patients. Cardiovascular pathology was found in 35.7%, and pulmonary at 63,8% patients. At the analysis of patients with systemic lupus erythematosus, 30.3% of them had renal involvement, manifested by proteinuria in 47.8% and hematuria in 31.3%. Central nervous system pathology was manifested by 78,5% headache and polyneuropthy in 24,6% of patients. Conclusions. Musculoskeletal manifestations are the most common in systemic lupus erythematosus. It is necessary to conduct investigations of the respiratory, cardiovascular, renal and nervous systems in systemic lupus erythematosus, at the early stages, for timely determination of the pathology and treatment.

Published
2020
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6. Use of platelet inhibitors for digital ulcers related to systemic sclerosis: EUSTAR study on derivation and validation of the DU-VASC model

Author

Alexandru Garaiman 1, Klaus Steigmiller 2, Catherine Gebhard 3, Carina Mihai 1, Rucsandra Dobrota 1, Cosimo Bruni 4, Marco Matucci-Cerinic 5, Joerg Henes 6, Jeska de Vries-Bouwstra 7, Vanessa Smith 8, Andrea Doria 9, Yannick Allanore 10, Lorenzo Dagna 11, Branimir Anić 12, Carlomaurizio Montecucco 13, Otylia Kowal-Bielecka 14, Mickael Martin 15, Yoshiya Tanaka 16, Anna-Maria Hoffmann-Vold 17, Ulrike Held 2, Oliver Distler 1, Mike Oliver Becker 1, EUSTAR Silvia Bellando Randone, Gemma Lepri, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Ewa Gindzienska-Sieskiewicz, Katarzyna Karaszewska, Maurizio Cutolo, Giovanna Cuomo, Elise Siegert, Simona Rednic, Jérome Avouac, Carole Desbas, Roberto Caporali, Lorenzo Cavagna, Patricia E Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J Kucharz, Elisabetta Zanatta, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstr, Mislav Radic, Alexandra Balbir-Gurman, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Nemanja Damjanov, Ann-Christian Pecher, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Ivan Foeldvari, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Lidia P Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Carlos de la Puente, Øyvind Midtvedt, Torhild Garen, Håvard Fretheim, Eric Hachulla, Valeria Riccieri, Ruxandra Maria Ionescu, Ana Maria Gheorghiu, Cord Sunderkötter, Jörg Distler, Francesca Ingegnoli, Luc Mouthon, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Marko Baresic, Miroslav Mayer, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Alessandra Vacca, Kamal Solanki, Douglas Veale, Esthela Loyo, Carmen Tineo, Mengtao Li, Edoardo Rosato, Fahrettin Oksel, Figen Yargucu, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Marzena Olesinska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianch, Breno Valdetaro Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M Hsu, Thierry Martin, Sergey Moiseev, Pavel Novikov, Lorinda S Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Gabriela Riemekasten, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Daniel Furst, Ana-Maria Ramazan, H U Scherer, Tom W J Huizinga, Marie-Elise Truchetet, Alain Lescoat, Giacomo De Luca, Corrado Campochiaro, J M van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Cédric L, Mathieu Puyade, Pascal Roblot, Satoshi Kubo, Yasuyuki Todoroki, 1, Alexandru Garaiman, 2, Klaus Steigmiller, 3, Catherine Gebhard, 1, Carina Mihai, 1, Rucsandra Dobrota, 4, Cosimo Bruni, 5, Marco Matucci-Cerinic, 6, Joerg Hene, 7, Jeska de Vries-Bouwstra, 8, Vanessa Smith, 9, Andrea Doria, Allanore 10, Yannick, Dagna 11, Lorenzo, Anić 12, Branimir, Montecucco 13, Carlomaurizio, Kowal-Bielecka 14, Otylia, Martin 15, Mickael, Tanaka 16, Yoshiya, Hoffmann-Vold 17, Anna-Maria, 2, Ulrike Held, 1, Oliver Distler, 1, Mike Oliver Becker, Silvia Bellando Randone, Eustar, Lepri, Gemma, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Gindzienska-Sieskiewicz, Ewa, Karaszewska, Katarzyna, Cutolo, Maurizio, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Avouac, Jérome, Desbas, Carole, Caporali, Roberto, Cavagna, Lorenzo, E Carreira, Patricia, Novak, Srdan, Czirják, László, Iudici, Michele, J Kucharz, Eugene, Zanatta, Elisabetta, Coleiro, Bernard, Moroncini, Gianluca, Farge Bancel, Dominique, Airò, Paolo, Hesselstr, Roger, Radic, Mislav, Balbir-Gurman, Alexandra, Hunzelmann, Nicola, Pellerito, Raffaele, Giollo, Alessandro, Morovic-Vergles, Jadranka, Denton, Christopher, Damjanov, Nemanja, Pecher, Ann-Christian, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Foeldvari, Ivan, João Salvador, Maria, Stamenkovic, Bojana, Francesco Selmi, Carlo, P Ananieva, Lidia, Herrick, Ariane, Müller-Ladner, Ulf, DE PALMA, Raffaele, Engelhart, Merete, Szücs, Gabriela, de la Puente, Carlo, Midtvedt, Øyvind, Garen, Torhild, Fretheim, Håvard, Hachulla, Eric, Riccieri, Valeria, Maria Ionescu, Ruxandra, Maria Gheorghiu, Ana, Sunderkötter, Cord, Distler, Jörg, Ingegnoli, Francesca, Mouthon, Luc, Paolo Cantatore, Francesco, Ullman, Susanne, Rosa Pozzi, Maria, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Baresic, Marko, Mayer, Miroslav, Yavuz, Sule, Granel, Brigitte, de Souza Müller, Carolina, Agachi, Svetlana, Stebbings, Simon, Mathieu, D'Alessandro, Vacca, Alessandra, Solanki, Kamal, Veale, Dougla, Loyo, Esthela, Tineo, Carmen, Li, Mengtao, Rosato, Edoardo, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Foti, Rosario, Ancuta, Codrina, Maurer, Britta, van Laar, Jacob, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Sibilia, Jean, Litinsky, Ira, Del Galdo, Francesco, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Bianch, Washington, Valdetaro Bianchi, Breno, Castellví, Ivan, Limonta, Massimiliano, Rimar, Doron, Couto, Maura, Spertini, Françoi, Marcoccia, Antonella, Kahl, Sarah, M Hsu, Ivien, Martin, Thierry, Moiseev, Sergey, Novikov, Pavel, S Chung, Lorinda, Schmeiser, Tim, Majewski, Dominik, Zdrojewski, Zbigniew, Martínez-Barrio, Julia, Bernardino, Vera, Riemekasten, Gabriela, Levy, Yair, Rezus, Elena, Nuri Pamuk, Omer, Sarzi Puttini, Piercarlo, Poormoghim, Hadi, Kötter, Ina, Gaches, Franci, Belloli, Laura, Sfikakis, Petro, Furst, Daniel, Ramazan, Ana-Maria, U Scherer, H, J Huizinga, Tom W, Truchetet, Marie-Elise, Lescoat, Alain, De Luca, Giacomo, Campochiaro, Corrado, M van Laar, J, Rudnicka, Lidia, Oliveira, Susana, Atzeni, Fabiola, Kuwana, Masataka, Mekinian, Arsene, L, Cédric, Puyade, Mathieu, Roblot, Pascal, Kubo, Satoshi, Todoroki, Yasuyuki, and University of Zurich

Subjects
prognostic prediction model, Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, digital ulcers, platelets inhibitors, 610 Medicine & health, Pharmacology (medical), SSc, 10060 Epidemiology, Biostatistics and Prevention Institute (EBPI)
Abstract

Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of digital ulcers (DUs). C-Statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C-statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C-statistic = 81.1% [95% CI: 78.9%, 83.4%] for the derivation and 82.3% [95% CI: 779.3%, 85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were lower modified Rodnan skin score, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to phosphodiesterase-5 inhibitor, prostacyclin analogues or endothelin receptor antagonists seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PI treatment was the most important therapy-related predictor associated with reduced DU occurrence.

Published
2022

7. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

Author

Elisabetta Zanatta 1, Dörte Huscher 2, Augusta Ortolan 1, Jérôme Avouac 3, Paolo Airò 4, Alexandra Balbir-Gurman 5, Elise Siegert 6, Marco Matucci Cerinic 7, Franco Cozzi 8, Gabriela Riemekasten 9, Anna-Maria Hoffmann-Vold 10, Oliver Distler 11, Armando Gabrielli 12, Stefan Heitmann 13, Nicolas Hunzelmann 14, Carlomaurizio Montecucco 15, Jadranka Morovic-Vergles 16, Camillo Ribi 17, Andrea Doria 1, Yannick Allanore 3, EUSTAR collaborators, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Eric Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Patricia E Carreira, Rosario Foti, Srdan Novak, Michele Iudici, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi, Lidia P Ananieva, Gabriela Szücs, Carlos de la Puente, Ruxandra Maria Ionescu, Jörg Distler, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz, Carolina de Souza Müller, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Ivan Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Michaela Kohm, Bojana Stamenkovic, 1, Elisabetta Zanatta, 2, Dörte Huscher, 1, Augusta Ortolan, 3, Jérôme Avouac, 4, Paolo Airò, 5, Alexandra Balbir-Gurman, 6, Elise Siegert, 7, Marco Matucci Cerinic, 8, Franco Cozzi, 9, Gabriela Riemekasten, Hoffmann-Vold 10, Anna-Maria, Distler 11, Oliver, Gabrielli 12, Armando, Heitmann 13, Stefan, Hunzelmann 14, Nicola, Montecucco 15, Carlomaurizio, Morovic-Vergles 16, Jadranka, Ribi 17, Camillo, 1, Andrea Doria, 3, Yannick Allanore, Collaborators, Eustar, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Hachulla, Eric, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, E Carreira, Patricia, Foti, Rosario, Novak, Srdan, Iudici, Michele, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi, Carlo, P Ananieva, Lidia, Szücs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Distler, Jörg, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz, Sule, de Souza Müller, Carolina, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Ivan, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Kohm, Michaela, and Stamenkovic, Bojana

Subjects
interstitial lung disease, Scleroderma, Systemic, Hypertension, Pulmonary, disease subset, anti-topoisomerase I, Scleroderma, Systemic sclerosis, cutaneous form, outcome, Phenotype, Rheumatology, Scleroderma, Limited, Antibodies, Antinuclear, Scleroderma, Diffuse, Humans, Pharmacology (medical), Lung Diseases, Interstitial
Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.

Published
2022

8. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database

Author

Iudici, Michele, Mongin, Denis, Siegert, Elise, Carreira, Patricia E, Distler, Jörg, Henes, Jörg, Zanatta, Elisabetta, Hachulla, Eric, De Luca, Giacomo, Müller, Carolina de Souza, Santiago, Tânia, Tandaipan, José-Luis, Bianchi, Breno Valdetaro, De Santis, Maria, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Courvoisier, Delphine Sophie, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Rosario Foti, Srdan Novak, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, Gabriela Szűcs, Carlos de la Puente, Ruxandra Maria Ionescu, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Iván Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Michaela Kohm, Bojana Stamenkovic, Yannick Allanore, Paolo Airo, Alexandra Balbir-Gurman, Marco Matucci Cerinic, Gabriela Riemekasten, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morovic-Vergles, Camillo Ribi, Michele, Iudici, Denis, Mongin, Elise, Siegert, Patricia E, Carreira, Jörg, Distler, Jörg, Hene, Elisabetta, Zanatta, Eric, Hachulla, Giacomo, De Luca, Carolina de Souza, Müller, Tânia, Santiago, José-Luis, Tandaipan, Breno Valdetaro, Bianchi, Maria, De Santi, Anna-Maria, Hoffmann-Vold, Armando, Gabrielli, Oliver, Distler, Courvoisier, Sophie, Delphine, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, Foti, Rosario, Novak, Srdan, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi Rozzano, Carlo, P Ananieva, Lidia, Szűcs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz Altunizade, Sule, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Iván, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Ortiz Santamaria Granollers, Vera, Kohm, Michaela, Stamenkovic, Bojana, Allanore, Yannick, Airo, Paolo, Balbir-Gurman, Alexandra, Matucci Cerinic, Marco, Riemekasten, Gabriela, Heitmann, Stefan, Hunzelmann, Nicola, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, and Ribi, Camillo

Subjects
Rheumatology, systemic sclerosis, Pharmacology (medical), epidemiology, glucocorticoid
Abstract

Objectives To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries. Methods We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P Conclusions GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed.

Published
2022

9. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Muriel Elhai, Nanthara Sritharan, Marouane Boubaya, Alexandra Balbir-Gurman, Elise Siegert, Eric Hachulla, Jeska de Vries-Bouwstra, Gabriela Riemekasten, Jörg H W Distler, Edoardo Rosato, Francesco Del Galdo, Fabian A Mendoza, Daniel E Furst, Carlos de la Puente, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Coralie Bloch-Queyrat, Yannick Allanore, Marco Matucci Cerinic, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Otylia Kowal Bielecka, Maurizio Cutolo, Giovanna Cuomo, Claudia Kedor, Simona Rednic, Jérome Avouac, P. Vlachoyiannopoulos, C. Montecucco, Jiri Stork, Murat Inanc, Patricia E. Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Katja Perdan-Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstrand, Mislav Radic, Yolanda Braun-Moscovici, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Madelon Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Kohm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Lidia P. Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Cristina Sobrino Grande, Øyvind Midtvedt, David Launay, Valeria Riccieri, Ruxandra Maria Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anic, Maria Üprus, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Joanna Busquets, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Percival D. Sampaio-Barros, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Antonietta Gigante, Fahrettin Oksel, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Giuseppina Abignano, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M. Hsu, Thierry Martin, Sergey Moiseev, Lorinda S. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Sabine Sommerlatte, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Juliana Markus, Gary R Feldman, Ana-Maria Ramazan, H.U. Scherer, Marie-Elise Truchetet, Alain Lescoat, Lorenzo Dagna, J.M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Mickaël Martin, Yoshiya Tanaka, Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., de Vries-Bouwstra, J., Riemekasten, G., Distler, J. H. W., Rosato, E., Del Galdo, F., Mendoza, F. A., Furst, D. E., de la Puente, C., Hoffmann-Vold, A. -M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., Matucci Cerinic, M., Walker, U., Iannone, F., Jordan, S., Becvar, R., Kowal Bielecka, O., Cutolo, M., Cuomo, G., Kedor, C., Rednic, S., Avouac, J., Vlachoyiannopoulos, P., Montecucco, C., Stork, J., Inanc, M., Carreira, P. E., Novak, S., Czirjak, L., Iudici, M., Kucharz, E. J., Zanatta, E., Perdan-Pirkmajer, K., Coleiro, B., Moroncini, G., Farge Bancel, D., Airo, P., Hesselstrand, R., Radic, M., Braun-Moscovici, Y., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Giollo, A., Morovic-Vergles, J., Denton, C., Vonk, M., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Hasler, P., Kohm, M., Foeldvari, I., Bajocchi, G., Salvador, M. J., Stamenkovic, B., Selmi, C. F., Tikly, M., Ananieva, L. P., Herrick, A., Muller-Ladner, U., De Palma, R., Engelhart, M., Szucs, G., Sobrino Grande, C., Midtvedt, O., Launay, D., Riccieri, V., Ionescu, R. M., Sha, A., Gheorghiu, A. M., Sunderkotter, C., Ingegnoli, F., Mouthon, L., Smith, V., Cantatore, F. P., Ullman, S., Alberto von Muhlen, C., Pozzi, M. R., Eyerich, K., Wiland, P., Vanthuyne, M., Alegre-Sancho, J. J., Herrmann, K., De Langhe, E., Anic, B., Uprus, M., Yavuz, S., Granel, B., de Souza Muller, C., Busquets, J., Agachi, S., Stebbings, S., Mathieu, D. A., Sampaio-Barros, P. D., Stamp, L., Solanki, K., Veale, D., Loyo, E., Li, M., Abdel Atty Mohamed, W. A., Gigante, A., Oksel, F., Tanaseanu, C. -M., Foti, R., Ancuta, C., Maurer, B., van Laar, J., Kayser, C., Fathi, N., Garcia de la Pena Lefebvre, P., Sibilia, J., Litinsky, I., Abignano, G., Seskute, G., Saketkoo, L. A., Kerzberg, E., Bianchi, W., Castellvi, I., Limonta, M., Rimar, D., Couto, M., Spertini, F., Marcoccia, A., Kahl, S., Hsu, I. M., Martin, T., Moiseev, S., Chung, L. S., Schmeiser, T., Majewski, D., Zdrojewski, Z., Martinez-Barrio, J., Bernardino, V., Sommerlatte, S., Levy, Y., Rezus, E., Nuri Pamuk, O., Sarzi Puttini, P., Poormoghim, H., Kotter, I., Gaches, F., Belloli, L., Sfikakis, P., Markus, J., Feldman, G. R., Ramazan, A. -M., Scherer, H. U., Truchetet, M. -E., Lescoat, A., Dagna, L., van Laar, J. M., Rudnicka, L., Oliveira, S., Atzeni, F., Kuwana, M., Mekinian, A., Martin, M., and Tanaka, Y.

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.

Published
2022

10. Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

Author

Allanore Y., Bozzi S., Terlinden A., Huscher D., Amand C., Soubrane C., Siegert E., Czirjak L., Carreira P. E., Hachulla E., Zanatta E., Li M., Airo P., Mendoza F. A., Rosato E., Distler O. Svetlana Agachi, Walid Ahmed Abdel Atty Mohamed, Paolo Airò, Sabine Adler, Juan Jose Alegre-Sancho, Yannick Allanore, Lidia P. Ananieva, Codrina Ancuta, Branimir Anic, Fabiola Atzeni, Gianluigi Bajocchi, Alexandra Balbir-Gurman, Marko Baresic, Radim Becvar, Laura Belloli, Vera Bernardino, Washington Bianchi, Breno Valdetaro Bianchi, Otylia Kowal Bielecka, Francesco Paolo Cantatore, Patricia E. Carreira, Ivan Castellví, Marco Matucci Cerinic, Carlo Chizzolini, Lorinda S. Chung, Bernard Coleiro, Maura Couto, Mary Ellen Csuka, Giovanna Cuomo, Maurizio Cutolo, László Czirják, Lorenzo Dagna, Nemanja Damjanov, Ellen De Langhe, Francesco Del Galdo, Carlos de la Puente, Christopher Denton, Y., Allanore, S., Bozzi, A., Terlinden, D., Huscher, C., Amand, C., Soubrane, E., Siegert, L., Czirjak, P. E., Carreira, E., Hachulla, E., Zanatta, M., Li, P., Airo, F. A., Mendoza, E., Rosato, Svetlana Agachi, Distler O., Ahmed Abdel Atty Mohamed, Walid, Airò, Paolo, Adler, Sabine, Jose Alegre-Sancho, Juan, Allanore, Yannick, Ananieva, Lidia P., Ancuta, Codrina, Anic, Branimir, Atzeni, Fabiola, Bajocchi, Gianluigi, Balbir-Gurman, Alexandra, Baresic, Marko, Becvar, Radim, Belloli, Laura, Bernardino, Vera, Bianchi, Washington, Valdetaro Bianchi, Breno, Kowal Bielecka, Otylia, Paolo Cantatore, Francesco, Carreira, Patricia E., Castellví, Ivan, Matucci Cerinic, Marco, Chizzolini, Carlo, Chung, Lorinda S., Coleiro, Bernard, Couto, Maura, Ellen Csuka, Mary, Cuomo, Giovanna, Cutolo, Maurizio, Czirják, László, Dagna, Lorenzo, Damjanov, Nemanja, De Langhe, Ellen, Del Galdo, Francesco, de la Puente, Carlo, Denton, Christopher, Publica, and University of Zurich

Subjects
0301 basic medicine, Longitudinal study, lcsh:Diseases of the musculoskeletal system, medicine.medical_treatment, 2745 Rheumatology, HAQ-DI score, computer.software_genre, Severity of Illness Index, 0302 clinical medicine, Quality of life, Surveys and Questionnaires, Medicine and Health Sciences, Longitudinal Studies, 610 Medicine & health, skin and connective tissue diseases, Database, Mortality rate, 10051 Rheumatology Clinic and Institute of Physical Medicine, EUSTAR registry, Diffuse cutaneous systemic sclerosis, Health Assessment Questionnaire-Disability Index, HAQ-DI score, 2723 Immunology and Allergy, Disease Progression, Hemodialysis, ARTHRITIS, CLINICAL-TRIALS, Research Article, musculoskeletal diseases, medicine.medical_specialty, Diffuse cutaneous systemic sclerosis, PREDICTING MORTALITY, EUSTAR registry, CLASSIFICATION, 03 medical and health sciences, Internal medicine, SCORE, medicine, Humans, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Proportional hazards model, Diffuse cutaneous systemic sclerosi, Health Assessment Questionnaire-Disability Index, Rheumatology, Clinical trial, 030104 developmental biology, Scleroderma, Diffuse, Quality of Life, FUNCTIONAL DISABILITY, Observational study, lcsh:RC925-935, Mitoxantrone, business, computer
Abstract

BackgroundPatients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.MethodsThis was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.ResultsThe analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p p p ConclusionsHAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

Published
2020

11. Significant weight loss in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author

Michael Hughes, Calvin Heal, Elise Siegert, Eric Hachulla, Paolo Airó, Antonella Riccardi, Oliver Distler, Marco Matucci-Cerinic, Andrea Doria, Lorenzo Baretta, Alexandra Balbir-Gurman, Patricia E Carreira, Vanessa Smith, Carlos Alberto, Jörg Distler von Mühlen, Ulf Müller-Ladner, Lidia P Ananieva, László Czirják, Jörg Henes, Jeska de Vries-Bouwstra, Mengtao Li, Fabian Mendoza, Nemanja Damjanov, Ivan Castellví, Alessandro Giollo, Stefan Heitmann, Edoardo Rosato, Lorenzo Dagna, Christopher P Denton, Marie Vanthuyne, Fabio Cacciapaglia, Valeria Riccieri, Nicolas Hunzelmann, Ami Shah, Carlomaurizio Montecucco, Raffaele Pellerito, Ruxandra Maria Ionescu, Simona Rednic, Ulrich Walker, Maria Rosa Pozzi, Anna-Maria Hoffmann-Vold, Marie-Elise Truchetet, Susanne Ullman, Carolina de Souza Müller, Juan Jose Alegre-Sancho, Eduardo Kerzberg, Francesco Del Galdo, Gabriela Riemekasten, Branimir Anic, Marko Baresic, Miroslav Mayer, Fahrettin Oksel, Figen Yargucu, Ellen De Langhe, Ina Kötter, Mohammed Tikly, Radim Becvar, Douglas Veale, Dorota Krasowska, Andrea Lo Monaco, Lidia Rudnicka, Ana Maria Gheorghiu, Piercarlo Sarzi Puttini, Mislav Radic, Armando Gabrielli, Maria João Salvador, Carlos de la Puente, Gabriela Szücs, Sule Yavuz, Rosario Foti, Otylia Kowal Bielecka, Codrina Ancuta, Peter Villiger, Sabine Adler, Patrick Jego, Michaela Kohm, Eugene J Kucharz, Dominique Farge Bancel, Tim Schmeiser, Alberto Cauli, Alessandra Vacca, Kamal Solanki, Piotr Wiland, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Sergio Jimenez, Lesley Ann Saketkoo, Roger Hesselstrand, Francesca Ingegnoli, Jean Sibilia, Merete Engelhart, Esthela Loyo, Carmen Tineo, Francesco Paolo Cantatore, Brigitte Krummel-Lorenz, Petros Sfikakis, Cristiane Kayser, Vera Ortiz Santamaria, Bojana Stamenkovic, Giovanna Cuomo, Francesco Puppo, Thierry Zenone, Nihal Fathi, Ira Litinsky, Carlo Chizzolini, Monika Swacha, Washington Bianchi, Breno Valdetaro Bianchi, Maria Üprus, Kati Otsa, Masataka Kuwana, Panayiotis Vlachoyiannopoulos, Sarah Kahl, Bernard Coleiro, François Spertini, Walid Ahmed Abdel Atty Mohamed, Sergey Moiseev, Pavel Novikov, Dominik Majewski, Simon Stebbings, Svetlana Agachi, Massimiliano Limonta, Carlo Francesco Selmi, Elena Rezus, Kristine Herrmann, Brigitte Granel, Goda Seskute, Matthias Seidel, Paul Hasler, Maurizio Cutolo Vera Bernardino, Carmen Pizzorni, Jadranka Morovic-Vergles, Daniel Furst, Ana-Maria Ramazan, Gianluigi Bajocchi, Lisa Stamp, Doron Rimar, Antonella Marcoccia, Srdan Novak, Luc Mouthon, Jiri Stork, Lorinda S Chung, Hadi Poormoghim, Francis Gaches, Laura Belloli, Cristina-Mihaela Tanaseanu, Fabiola Atzeni, Kilian Eyerich, Ivien M Hsu, Jacob van Laar, Mary Ellen Csuka, Omer Nuri Pamuk, Maura Couto, Arsene Mekinian, Murat Inanc, Ivan Foeldvari, Julia Martínez-Barrio, Yair Levy, Juliana Markus, Susana Oliveira, Hughes, Michael, Heal, Calvin, Siegert, Elise, Hachulla, Eric, Airó, Paolo, Riccardi, Antonella, Distler, Oliver, Matucci-Cerinic, Marco, Doria, Andrea, Baretta, Lorenzo, Balbir-Gurman, Alexandra, E Carreira, Patricia, Smith, Vanessa, Alberto, Carlo, Distler von Mühlen, Jörg, Müller-Ladner, Ulf, P Ananieva, Lidia, Czirják, László, Henes, Jörg, de Vries-Bouwstra, Jeska, Li, Mengtao, Mendoza, Fabian, Damjanov, Nemanja, Castellví, Ivan, Giollo, Alessandro, Heitmann, Stefan, Rosato, Edoardo, Dagna, Lorenzo, P Denton, Christopher, Vanthuyne, Marie, Cacciapaglia, Fabio, Riccieri, Valeria, Hunzelmann, Nicola, Shah, Ami, Montecucco, Carlomaurizio, Pellerito, Raffaele, Maria Ionescu, Ruxandra, Rednic, Simona, Walker, Ulrich, Rosa Pozzi, Maria, Hoffmann-Vold, Anna-Maria, Truchetet, Marie-Elise, Ullman, Susanne, de Souza Müller, Carolina, Jose Alegre-Sancho, Juan, Kerzberg, Eduardo, Del Galdo, Francesco, Riemekasten, Gabriela, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Oksel, Fahrettin, Yargucu, Figen, De Langhe, Ellen, Kötter, Ina, Tikly, Mohammed, Becvar, Radim, Veale, Dougla, Krasowska, Dorota, Lo Monaco, Andrea, Rudnicka, Lidia, Maria Gheorghiu, Ana, Sarzi Puttini, Piercarlo, Radic, Mislav, Gabrielli, Armando, João Salvador, Maria, de la Puente, Carlo, Szücs, Gabriela, Yavuz, Sule, Foti, Rosario, Kowal Bielecka, Otylia, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Jego, Patrick, Kohm, Michaela, J Kucharz, Eugene, Farge Bancel, Dominique, Schmeiser, Tim, Cauli, Alberto, Vacca, Alessandra, Solanki, Kamal, Wiland, Piotr, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Jimenez, Sergio, Ann Saketkoo, Lesley, Hesselstrand, Roger, Ingegnoli, Francesca, Sibilia, Jean, Engelhart, Merete, Loyo, Esthela, Tineo, Carmen, Paolo Cantatore, Francesco, Krummel-Lorenz, Brigitte, Sfikakis, Petro, Kayser, Cristiane, Ortiz Santamaria, Vera, Stamenkovic, Bojana, Cuomo, Giovanna, Puppo, Francesco, Zenone, Thierry, Fathi, Nihal, Litinsky, Ira, Chizzolini, Carlo, Swacha, Monika, Bianchi, Washington, Valdetaro Bianchi, Breno, Üprus, Maria, Otsa, Kati, Kuwana, Masataka, Vlachoyiannopoulos, Panayioti, Kahl, Sarah, Coleiro, Bernard, Spertini, Françoi, Ahmed Abdel Atty Mohamed, Walid, Moiseev, Sergey, Novikov, Pavel, Majewski, Dominik, Stebbings, Simon, Agachi, Svetlana, Limonta, Massimiliano, Francesco Selmi, Carlo, Rezus, Elena, Herrmann, Kristine, Granel, Brigitte, Seskute, Goda, Seidel, Matthia, Hasler, Paul, Cutolo Vera Bernardino, Maurizio, Pizzorni, Carmen, Morovic-Vergles, Jadranka, Furst, Daniel, Ramazan, Ana-Maria, Bajocchi, Gianluigi, Stamp, Lisa, Rimar, Doron, Marcoccia, Antonella, Novak, Srdan, Mouthon, Luc, Stork, Jiri, S Chung, Lorinda, Poormoghim, Hadi, Gaches, Franci, Belloli, Laura, Tanaseanu, Cristina-Mihaela, Atzeni, Fabiola, Eyerich, Kilian, M Hsu, Ivien, van Laar, Jacob, Ellen Csuka, Mary, Nuri Pamuk, Omer, Couto, Maura, Mekinian, Arsene, Inanc, Murat, Foeldvari, Ivan, Martínez-Barrio, Julia, Levy, Yair, Markus, Juliana, and Oliveira, Susana

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, weight loss, systemic sclerosis, nutrition, Immunology, Disease, computer.software_genre, General Biochemistry, Genetics and Molecular Biology, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Weight loss, Internal medicine, Weight Loss, Epidemiology, medicine, Humans, Immunology and Allergy, 610 Medicine & health, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, Database, business.industry, Middle Aged, medicine.disease, 030104 developmental biology, Mood, Databases as Topic, Female, Outcomes research, medicine.symptom, business, computer, Rheumatism
Abstract

Gastrointestinal (GI) involvement is almost universal in patients with systemic sclerosis (SSc) and is associated with significant disease-related morbidity and mortality.1 The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease.2 However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc. Therefore, there is a need to understand high-risk patients including potentially modifiable risk factors, with a view to early intervention strategies. Against this background, the aim of this study was to examine potential clinical risk factors of significant weight loss in patients with SSc. We performed an analysis of patients with SSc enrolled in the multinational, longitudinal European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) database. In our study, we defined significant weight loss as 4.5 kg and/or least 5% of their body weight at 5 months onwards.3 Patients with a recorded second visit after 3 months and before 12 months were included in the analysis. We adopted a pragmatic approach (relevant to clinical practice) in …

Published
2020
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12. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author

Antonio C. Zea Mendoza, Jean Sibilia, Kamal Solanki, Cristina Mihaela Tanaseanu, Fredrick M. Wigley, Guido Valesini, M. Govoni, Lisa K. Stamp, Christopher P. Denton, Yolanda Braun-Moscovici, Ruxandra Ionescu, Øyvind Midtvedt, Ileana Nicoara, Aleksandra Stanković, Rüdiger Hein, Alina Dumitrascu, Susanne Ullman, Alan Tyndall, Sergio A. Jimenez, Irena Butrimiene, Alan Doube, Eugene J. Kucharz, Mohammed Tikly, Pier Luigi Meroni, Simon Stebbings, Renata Sokolik, Alexandra Balbir Gurman, Roger Hesselstrand, Kirsten Damgaard, Francesco Paolo Cantatore, Razvan Ionitescu, Silvana Zeni, Marco Matucci-Cerinic, Maria Rosa Pozzi, Jacques-Eric Gottenberg, Srdan Novak, Ana Maria Gherghe, David Launay, Liliana Groppa, Carlomaurizio Montecucco, Roxana Sfrent Cornateanu, Stefan Heitmann, Paloma García de la Peña Lefebvre, Daniela Opris, Peter T. Chapman, Line V. Iversen, Bernard Coleiro, Ulf Müller-Ladner, John Highton, Mara Oleszowsky, Gabriella Szücs, Magdalena Kopec-Medrek, Carlos De La Puente Buijdos, Paola Caramaschi, Magdalena Szmyrka-Kaczmarek, Rucsandra Dobrota, Gabriele Valentini, Fabiana Montoya, Blaz Rozman, Alberto Sulli, Hélène Chifflot, Raffaella Scorza, Patricia Carreira, Paulius Venalis, Lisa Maria Bambara, Torhild Garen, Isabela Tiglea, Agneta Scheja, Duska Martinovic, Jörg H W Distler, Jörg Henes, Giovanni Lapadula, Luc Mouthon, Diana Karpec, Douglas J. Veale, Valeria Riccieri, Nicolas Hunzelmann, Muriel Elhai, Serena Guiducci, Codrina Ancuta, Simonetta Pisarri, Thierry Zenone, Esthela Loyo, Branimir Anić, Claudia Günther, R. Becvar, Eugen Russu, Serena Vettori, Carlo Chizzolini, Vanessa Smith, Mengtao Li, Stanislaw Sierakowsky, Carmel Mallia, Małgorzata Widuchowska, Carolina de Souza Müller, László Czirják, Algirdas Venalis, Adrian Hij, Marta Valero Exposito, Simona Rednic, Miroslav Mayer, Laura Groseanu, Walter Alberto Sifuentes Giraldo, Murray Baron, Dominique Farge, Anna Kotulska, Marko Baresic, Svetlana Agachi, Martin Aringer, Merete Engelhart, John L. O'Donnell, Jérôme Avouac, Filip De Keyser, Ulrich A. Walker, Roberto Caporali, Harald Burkhardt, P. G. Vlachoyiannopoulos, Maurizio Cutolo, Frank A. Wollheim, Edoardo Rosato, Suzanne Kafaja, Valderílio Feijó Azevedo, Kilian Eyerich, Paolo Airò, Emmanuel Chatelus, L. Ananieva, Ira Litinsky, Andrea Lo Monaco, Vanesa Cosentino, Rita Rugiene, Eric Hachulla, P. Saar, Bojana Stamenkovic, Brigitte Krummel-Lorenz, Yannick Allanore, Elisabeth Knott, Oliver Distler, Matthias Seidel, Silvia Rodriguez Rubio, Franco Cozzi, Mihai Bojinca, Nemanja Damjanov, Maria João Salvador, Joanna Busquets, Otylia Kowal Bielecka, André Kahan, Jacek Szechiński, Daniel E. Furst, C. Mihai, Rodica Chirieac, Ewa Morgiel, Georg Schett, Armando Gabrielli, Giovanna Cuomo, Piotr Wiland, Maya N. Starovoytova, Sebastião Cezar Radominski, Gitte Strauss, Lealea Chiaburu, Florenzo Iannone, Carol M. Black, Andrea Himsel, Eduardo Kerzberg, Cecília Varjú, Vera Ortiz Santamaria, Gabriela Riemekasten, Dorota Krasowska, Marilena Gorga, Monica Popescu, Marie O'Rourke, Henrik Nielsen, Raffaele Pellerito, Ada Corrado, Elhai, M, Avouac, J, Walker, Ua, Matucci Cerinic, M, Riemekasten, G, Airò, P, Hachulla, E, Valentini, Gabriele, Carreira, Pe, Cozzi, F, Balbir Gurman, A, Braun Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller Ladner, U, Kahan, A, Distler, O, Allanore, Y, EUSTAR co, Author, EUSTAR co, Authors, Matucci-Cerinic, M, Airo, P, Valentini, G, Gurman, Ab, Braun-Moscovici, Y, Mt, Li, Muller-Ladner, U, Allanore, Y EUSTAR co-authors: Serena Guiducci, Alan, Tyndall, Giovanni, Lapadula, Florenzo, Iannone, Radim, Becvar, Stanislaw, Sierakowsky, Otylia Kowal Bielecka, Maurizio, Cutolo, Alberto, Sulli, Cuomo, Giovanna, Vettori, Serena, Simona, Rednic, Ileana, Nicoara, Vlachoyiannopoulos, P, Montecucco, C, Roberto, Caporali, Srdan, Novak, László, Czirják, Cecilia, Varju, Carlo, Chizzolini, Eugene, J Kucharz, Anna, Kotulska, Magdalena, Kopec-Medrek, Malgorzata, Widuchowska, Blaz, Rozman, Carmel, Mallia, Bernard, Coleiro, Armando, Gabrielli, Dominique, Farge, Adrian, Hij, Roger, Hesselstrand, Agneta, Scheja, Frank, Wollheim, Duska, Martinovic, Govoni, M, Andrea Lo Monaco, Nicolas, Hunzelmann, Raffaele, Pellerito, Lisa Maria Bambara, Paola, Caramaschi, Carol, Black, Christopher, Denton, Jörg, Hene, Vera Ortiz Santamaria, Stefan, Heitmann, Dorota, Krasowska, Matthias, Seidel, Mara, Oleszowsky, Harald, Burkhardt, Andrea, Himsel, Maria, J Salvador, Bojana, Stamenkovic, Aleksandra, Stankovic, Mohammed, Tikly, Maya, N Starovoytova, Merete, Engelhart, Gitte, Strau, Henrik, Nielsen, Kirsten, Damgaard, Gabriella, Szüc, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter, A Sifuentes Giraldo, Øyvind, Midtvedt, Torhild, Garen, David, Launay, Guido, Valesini, Valeria, Riccieri, Ruxandra Maria Ionescu, Daniela, Opri, Laura, Groseanu, Fredrick, M Wigley, Carmen, M Mihai, Roxana Sfrent Cornateanu, Razvan, Ionitescu, Ana Maria Gherghe, Marilena, Gorga, Rucsandra, Dobrota, Mihai, Bojinca, Georg, Schett, Jörg Hw Distler, Pierluigi, Meroni, Silvana, Zeni, Luc, Mouthon, Filip De Keyser, Vanessa, Smith, Francesco, P Cantatore, Ada, Corrado, Susanne, Ullman, Line, Iversen, Maria, R Pozzi, Kilian, Eyerich, Rüdiger, Hein, Elisabeth, Knott, Jacek, Szechinski, Piotr, Wiland, Magdalena, Szmyrka-Kaczmarek, Renata, Sokolik, Ewa, Morgiel, Brigitte, Krummel-Lorenz, Petra, Saar, Martin, Aringer, Claudia, Günther, Branimir, Anic, Marko, Baresic, Miroslav, Mayer, Sebastião, C Radominski, Carolina de Souza Müller, Valderílio, F Azevedo, Svetlana, Agachi, Liliana, Groppa, Lealea, Chiaburu, Eugen, Russu, Thierry, Zenone, Simon, Stebbing, John, Highton, Lisa, Stamp, Peter, Chapman, Murray, Baron, John, O'Donnell, Kamal, Solanki, Alan, Doube, Douglas, Veale, Marie, O'Rourke, Esthela, Loyo, Edoardo, Rosato, Simonetta, Pisarri, Cristina-Mihaela, Tanaseanu, Monica, Popescu, Alina, Dumitrascu, Isabela, Tiglea, Rodica, Chirieac, Codrina, Ancuta, Daniel, E Furst, Suzanne, Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean, Sibilia, Emmanuel, Chatelu, Jacques Eric Gottenberg, Hélène, Chifflot, Ira, Litinsky, Algirdas, Venali, Irena, Butrimiene, Paulius, Venali, Rita, Rugiene, Diana, Karpec, Eduardo, Kerzberg, Fabiana, Montoya, Vanesa, Cosentino, and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, heart dysfunction, Databases, Factual, Epidemiology, autoimmune diseases, epidemiology, systemic sclerosis, Kaplan-Meier Estimate, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Prospective Studies, Age of Onset, skin and connective tissue diseases, Prospective cohort study, ddc:616, Orvostudományok, Middle Aged, Prognosis, Connective tissue disease, 3. Good health, Europe, Cardiovascular Diseases, Cohort, Disease Progression, Female, Autoimmune Diseases, Systemic Sclerosis, Human, Adult, medicine.medical_specialty, Prognosi, Immunology, Socio-culturale, Klinikai orvostudományok, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, 03 medical and health sciences, Sex Factors, Rheumatology, Internal medicine, medicine, Humans, Sex Distribution, Systemic Sclerosi, Aged, 030203 arthritis & rheumatology, Lupus erythematosus, Scleroderma, Systemic, business.industry, medicine.disease, Pulmonary hypertension, Prospective Studie, 030104 developmental biology, Heart failure, Age of onset, business, Follow-Up Studies
Abstract

OBJECTIVES: In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. METHOD: We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. RESULTS: 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p

Published
2014

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