Introduction: Survival of scleroderma has changed since the treatment of renal crisis became possible. This study documents the changes in survival and causes of death in SSc over the past 25 years.
Methods: Patients studied were consecutive patients evaluated at the University of Pittsburgh between January 1, 1972 and December 31, 1996. Survival was determined in five 5 year time periods between 1972 and 1997. Causes of death included scleroderma related ones (Scleroderma Renal Crisis, Pulmonary Arterial Hypertension, Pulmonary Fibrosis, Gastrointestinal, Heart, and Multiorgan failure and non- scleroderma related deaths (cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown.
Results: There was a significant improvement in the 10 year survival for the 1982-1991 patients compared with the 1972-1981 patients (55% to 65%, p<0.001) even when renal crisis patients were excluded (p<0.005). The frequency of renal crisis deaths dramatically decreased over the 30 year time period, from 42% to 6% of scleroderma related deaths ( p<0.0001) while the proportion of scleroderma patients who died of pulmonary fibrosis increased from 6% to 33%(p<0.001). There were no changes in scleroderma gastrointestinal and cardiac deaths or in the non scleroderma related causes.
Discussion: The change in pattern of scleroderma- related mortality over the past 30 years implicates the lung (both pulmonary hypertension and pulmonary fibosis) as the primary causes of scleroderma related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of systemic sclerosis.
- pulmonary fibrosis
- systemic sclerosis