Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Issue 10 (15th June 2010)

• Record Type: Journal Article
• Authors: Tyndall, Anthony J; Bannert, Bettina; Vonk, Madelon; Airò, Paolo; Cozzi, Franco; Carreira, Patricia E; Bancel, Dominique Farge; Allanore, Yannick; Müller-Ladner, Ulf; Distler, Oliver; Iannone, Florenzo; Pellerito, Raffaele; Pileckyte, Margarita; Miniati, Irene; Ananieva, Lidia; Gurman, Alexandra Balbir; Damjanov, Nemanja; Mueller, Adelheid; Valentini, Gabriele; Riemekasten, Gabriela; Tikly, Mohammed; Hummers, Laura; Henriques, Maria JS; Caramaschi, Paola; Scheja, Agneta; Rozman, Blaz; Ton, Evelien; Kumánovics, Gábor; Coleiro, Bernard; Feierl, Eva; Szucs, Gabriella; Von Mühlen, Carlos Alberto; Riccieri, Valeria; Novak, Srdan; Chizzolini, Carlo; Kotulska, Anna; Denton, Christopher; Coelho, Paulo C; Kötter, Ina; Simsek, Ismail; de la Pena Lefebvre, Paloma García; Hachulla, Eric; Seibold, James R; Rednic, Simona; Štork, Jiří; Morovic-Vergles, Jadranka; Walker, Ulrich A;
• Is Part Of: Annals of the rheumatic diseases. Volume 69:Issue 10(2010)
• Page Start: 1809
• Page End: 1815
• Publication Date: 2010-06-15
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2009.114264
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan–Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). Conclusion: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.