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

László Czirják ^1*, Angéla Pákozdi ², Gábor Kumánovics ¹, Cecília Varjú ¹, Zoltán Szekanecz ², Zoltán Nagy ² and Gabriella Szûcs ²

¹ University of Pécs, Hungary
² University of Debrecen, Hungary

^* To whom correspondence should be addressed. E-mail: laszlo.czirjak{at}aok.pte.hu.

Accepted 7 May 2007

Abstract

Objective. Survival analysis of a series of 366 consecutive patients with systemic sclerosis (SSc).

Design. Clinical and laboratory data were evaluated since 1983 until 2005 by a standard protocol.

Subjects. 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 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 bad independent prognostic signs. Elderly age at the onset of disease also caused an unfavourable outcome. 86 SSc-related deaths were recorded during the follow-up. 65% of the deaths 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.

Keywords: Prognosis, malignancy, scleroderma, survival, systemic sclerosis

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