Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.
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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 J S, 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, Stork Jirí, Morovic-Vergles Jadranka, Walker Ulrich A
Аннотация
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.