Changes in causes of death in systemic sclerosis, 1972-2002

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.