Article Text

other Versions

Survival and causes of death in 366 Hungarian patients with systemic sclerosis
  1. László Czirják (laszlo.czirjak{at}
  1. University of Pécs, Hungary
    1. Angéla Pákozdi
    1. University of Debrecen, Hungary
      1. Gábor Kumánovics (gabor.kumanovics{at}
      1. University of Pécs, Hungary
        1. Cecília Varjú (cecilia.varju{at}
        1. University of Pécs, Hungary
          1. Zoltán Szekanecz (szekanecz{at}
          1. University of Debrecen, Hungary
            1. Zoltán Nagy (zoltan.nagy{at}
            1. University of Debrecen, Hungary
              1. Gabriella Szûcs (szucsg{at}
              1. University of Debrecen, Hungary


                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.

                • Prognosis
                • malignancy
                • scleroderma
                • survival
                • systemic sclerosis

                Statistics from

                Request permissions

                If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.