Carina Mihai1, Robert Landewé2, Désirée van der Heijde3, Ulrich A Walker4, Paul I Constantin1, Ana Maria Gherghe1, Ruxandra Ionescu5, Simona Rednic6, Yannick Allanore7, Jérôme Avouac7, László Czirják8, Eric Hachulla9, Gabriela Riemekasten10, Franco Cozzi11, Paolo Airò12, Maurizio Cutolo13, Ulf Mueller-Ladner14, Marco Matucci-Cerinic15. 1. Department of Internal Medicine and Rheumatology, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. 2. Amsterdam Rheumatology Center/University of Amsterdam & Atrium Medical Center, Heerlen, The Netherlands. 3. Leiden University Medical Center, Leiden, The Netherlands. 4. Basel University Hospital, Basel, Switzerland. 5. Department of Internal Medicine and Rheumatology, Sf. Maria Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania. 6. Rheumatology Clinic, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania. 7. Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France. 8. Department of Rheumatology and Immunology, University of Pecs, Pecs, Hungary. 9. Internal Medicine Department, Claude Huriez Hospital, Lille, France. 10. Department of Rheumatology, Charité University Hospital, Berlin, Germany. 11. Cattedra di Reumatologia, Dip. Medicina Clinica e Sperimentale, Policlinico, University of Padova, Padova, Italy. 12. Spedali Civili di Brescia, Rheumatology and Clinical Immunology Service, Brescia, Italy. 13. University of Genova, Research Laboratory and Academic Clinical Unit of Rheumatology, Genova, Italy. 14. Department of Rheumatology and Clinical Immunology, Giessen University, Bad Nauheim, Germany. 15. Division of Rheumatology AOUC & Department of Experimental and Clinical Medicine, Department of Biomedicine, University of Florence, Florence, Italy.
Abstract
OBJECTIVE: Systemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc. METHODS: Patients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3 years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis. RESULTS: 3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), p<0.001, for an elevated systolic pulmonary arterial pressure on heart ultrasound (US-PAPs):1.36 (1.03 to 1.80), p=0.032, for any cardiovascular event (new DUs, elevated US-PAPs or LV failure): 3.56 (2.26 to 5.62), p<0.001, and for death (1.53 (1.16 to 2.02), p=0.003). CONCLUSIONS: In patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
OBJECTIVE:Systemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc. METHODS:Patients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3 years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis. RESULTS: 3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), p<0.001, for an elevated systolic pulmonary arterial pressure on heart ultrasound (US-PAPs):1.36 (1.03 to 1.80), p=0.032, for any cardiovascular event (new DUs, elevated US-PAPs or LV failure): 3.56 (2.26 to 5.62), p<0.001, and for death (1.53 (1.16 to 2.02), p=0.003). CONCLUSIONS: In patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
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