Mortality in Systemic Sclerosis (Scleroderma)

Two hundred and thirty-seven patients with systemic sclerosiswere followed prospectively in a scleroderma clinic. The overall3, 6, and 9-year survival rates were 86, 76 and 61 per centrespectively. Renal, cardiac and pulmonary disease, and olderage at enrolment were adverse prognostic factors associatedwith reduced survival. There were no significant differencesin survival between males and females or in patients with restrictedcompared to those with diffuse skin thickening. Death from systemicsclerosis was most frequently due to pulmonary hypertension,with fewer than expected deaths from renal or cardiac causes.Twenty-eight per cent of deaths were due to causes unrelatedto systemic sclerosis, most commonly cancer and ischaemic heartdisease, and in older patients