Survival and causes of death in 366 Hungarian patients with systemic sclerosis

Objective: Survival analysis of a series of 366 consecutive patients with systemic sclerosis (SSc). Methods: Clinical and laboratory data were evaluated from 1983 until 2005 using a standard protocol. The female/male ratio was 315/51. The mean (SD) age of the patients was 56.8 (12.2) years. The duration of disease was 12 (5-19) years with a median follow-up of 6 (3-12) years. Results: Kaplan-Meier univariate analysis showed that renal, cardiac involvement, pigmentation disturbances, malabsorption, a forced vital capacity <50%, diffuse scleroderma, presence of early malignancy, anaemia, and increased erythrocyte sedimentation rate (ESR) were signs of unfavourable prognosis, whereas anti-centromere antibodies were indicators of a good survival. In the multivariate Cox proportional hazards model the presence of diffuse scleroderma, renal involvement, coexistence of a malignant disease, and increased ESR were poor independent prognostic signs. Elderly age at the onset of disease also caused an unfavourable outcome. A total of 86 SSc-related deaths were recorded during the follow-up. Of them, 65% were attributed to cardiorespiratory manifestation of disease. Tumour associated early death was found in 12 cases (14%). Conclusions: In addition to the well-known factors influencing the outcome (diffuse subset, internal organ involvements, and inflammatory signs), the coexistence of scleroderma with a malignancy also causes a poor outcome.