[2009] [OP-0078] CAUSES AND RISK FACTORS FOR DEATH IN SYSTEMIC SCLEROSIS – A STUDY FROM THE EULAR SCLERODERMA TRIALS AND RESEARCH (EUSTAR) DATA BASE
A.J. Tyndall1, B. Bannert1, M.C. Vonk2, P. Airò3, G. Riemekasten4, F. Cozzi5, P.E. Carreira6, D. Farge-Bancel7, Y. Allanore8, M. Mattuci Cerinic9, U.A. Walker1 1Rheumatology, Felix Platter Hospital, University Hospital Basel, Basel, Switzerland; 2Rheumatology, Radboud University Medical Centre Nijmegen, Nijmegen, Netherlands; 3Rheumatology, Spedali Civili di Brescia, Brescia, Italy; 4Rheumatology and Clinical Immunology, Charité-Universitätsmedizin, Berlin, Germany; 5Rheumatology, University of Padova, Padova, Italy; 6Rheumatology, Hospital 12 de Octubre, Madrid, Spain; 7Department of Internal Medicine, Hopital Saint-Louis; 8Rheumatology A, Cochin Hospital, Paris, France; 9Rheumatology, University of Florence, Florence, Italy
Background: Systemic sclerosis (SSc) is associated with a high mortality, but data concerning actual cause of death are often incomplete.
Objectives: To determine the causes and predictors of mortality in patients with SSc in a large multinational cohort.
Methods: Patients fulfilling the ACR criteria for SSc (n= 5860) and prospectively followed in the EULAR Scleroderma Trials And Research (EUSTAR) cohort were analyzed. EUSTAR centres registering patients as deceased (n=301) completed an extensive questionnaire on the cause of death and comorbidity.
Results: Responses were obtained on 234 fatalities from 45 centres. 55% of deaths were attributed directly to SSc, 41% to non-SSc causes; in 4% of cases, the cause mortality could not be assigned. Of the SSc related deaths, 35% were due to pulmonary fibrosis, and 26% to isolated pulmonary hypertension (PAH). Cardiac SSc related causes (mainly heart failure and arrhythmias) accounted for 26% of deaths. Of the non-SSc related causes, infections (33%) and malignancies (31%) were most common, followed by cardiovascular (29%) causes, mainly myocardial infarction, heart failure and stroke. Among the non-SSc related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis, and gastrointestinal haemorrhage).
Using Cox regression modelling, independent predictors of mortality were low diffusing capacity of the lung for carbon monoxide (DLCO, hazard ratio (HR) 1.20 per 10% decline), high modified Rodnan skin score (HR 1.02 per score point), higher age at Raynaud's onset (HR 1.03 per year), as well as the presence of pulmonary hypertension (HR 2.02), pulmonary restriction (HR 1.64), dyspnoea (HR 1.61) and proteinuria (HR 3.34). Clinical SSc subtype (diffuse vs. limited cutaneous SSc), autoantibody status and the presence of digital ulcers were not independent risk factors.
Conclusion: Pulmonary fibrosis and isolated PAH are pre-eminent causes of death in SSc. In addition, causes of death such as septicaemia and pneumonia could be directly related to SSc-associate comorbidity in many cases.
Other independent predictors of death included DLCO, pulmonary hypertension, modified Rodnan skin score, age at Raynaud's onset, dyspnoea and proteinuria.
Disclosure of Interest: None declared
Ann Rheum Dis 2009;68(Suppl3):95
Abstract Session: Systemic sclerosis from bench to bedside
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