Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort. Issue 5 (9th March 2019)

• Record Type: Journal Article
• Authors: Wu, Wanlong; Jordan, Suzana; Graf, Nicole; de Oliveira Pena, Janethe; Curram, John; Allanore, Yannick; Matucci-Cerinic, Marco; Pope, Janet E; Denton, Christopher P; Khanna, Dinesh; Distler, Oliver
• Other Names: author non-byline.; Matucci-Cerinic Marco author non-byline.; Guiducci Serena author non-byline.; Walker Ulrich author non-byline.; Jaeger Veronika author non-byline.; Bannert Bettina author non-byline.; Lapadula Giovanni author non-byline.; Becvarare Radim author non-byline.; Cutolo Maurizio author non-byline.; Valentini Gabriele author non-byline.; Siegert Elise author non-byline.; Rednic Simona author non-byline.; Allanore Yannick author non-byline.; Montecucco C. author non-byline.; Carreira Patricia E. author non-byline.; Novak Srdan author non-byline.; Czirják László author non-byline.; Varju Cecilia author non-byline.; Chizzolini Carlo author non-byline.; Allai Daniela author non-byline.; Kucharz Eugene J. author non-byline.; Cozzi Franco author non-byline.; Rozman Blaz author non-byline.; Mallia Carmel author non-byline.; Gabrielli Armando author non-byline.; Bancel Dominique Farge author non-byline.; Airò Paolo author non-byline.; Hesselstrand Roger author non-byline.; Martinovic Duska author non-byline.; Balbir-Gurman Alexandra author non-byline.; Braun-Moscovici Yolanda author non-byline.; Hunzelmann Nicolas author non-byline.; Pellerito Raffaele author non-byline.; Mauriziano Ospedale author non-byline.; Caramaschi Paola author non-byline.; Black Carol author non-byline.; Damjanov Nemanja author non-byline.; Henes Jörg author non-byline.; Santamaria Vera Ortiz author non-byline.; Heitmann Stefan author non-byline.; Seidel Matthias author non-byline.; Pereira Da Silva José Antonio author non-byline.; Stamenkovic Bojana author non-byline.; Selmi Carlo Francesco author non-byline.; Tikly Mohammed author non-byline.; Denisov Lev N. author non-byline.; Müller-Ladner Ulf author non-byline.; Engelhart Merete author non-byline.; Hachulla Eric author non-byline.; Riccieri Valeria author non-byline.; Ionescu Ruxandra Maria author non-byline.; Mihai Carina author non-byline.; Sunderkötter Cord author non-byline.; Kuhn Annegret author non-byline.; Schett Georg author non-byline.; 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• Is Part Of: Annals of the rheumatic diseases. Volume 78:Issue 5(2019)
• Page Start: 648
• Page End: 656
• Publication Date: 2019-03-09
• Subjects: diffuse cutaneous systemic sclerosis -- progressive skin fibrosis -- visceral organ progression -- lung function decline -- all-cause death; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-213455
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results: Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). Conclusions: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.