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  • Title: Changes in causes of death in systemic sclerosis, 1972-2002.
    Author: Steen VD, Medsger TA.
    Journal: Ann Rheum Dis; 2007 Jul; 66(7):940-4. PubMed ID: 17329309.
    Abstract:
    BACKGROUND: Survival of scleroderma has changed since the renal crisis treatment has become possible. AIMS: To document the changes in survival and organ system causes of mortality in systemic sclerosis (SSc) over the past 25 years in patients from a single medical centre. METHODS: Consecutive patients evaluated at the University of Pittsburgh, Pittsburgh, Pennsylvania, USA between 1 January 1972 and 31 December 1996 were studied. Survival was determined in five 5-year time periods between 1972 and 1997. Causes of death included scleroderma-related (scleroderma renal crisis, pulmonary arterial hypertension, pulmonary fibrosis (PF), gastrointestinal (GI), heart and multiorgan failure) and non-scleroderma-related (cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown) causes. RESULTS: The 10-year survival improved steadily from 54% to 66% during each of the time intervals. There was a significant improvement in survival for patients during 1982-91 compared with those during 1972-81 (p<0.001), even when patients with renal crisis were excluded (p<0.005). The frequency of deaths due to renal crisis significantly decreased over the 30-year time period, from 42% to 6% of scleroderma-related deaths (p<0.001), whereas the proportion of patients with scleroderma who died of PF increased from 6% to 33% (p<0.001). The frequency of pulmonary hypertension, independent of PF, also significantly increased during this time period (p<0.05). There were no changes in scleroderma GI- and heart-related deaths, nor in any of the non-scleroderma-related causes, although patients with scleroderma were less likely to die from scleroderma-related problems in the past 15 years. CONCLUSION: The change in the pattern of scleroderma-related deaths over the past 30 years demonstrates that the lung (both pulmonary hypertension and PF) is the primary cause of scleroderma-related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of SSc.
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