Although pulmonary disease is now the main cause of mortality in systemic sclerosis, there is a subset of patients with early rapidly progressive disease and a high risk of renal and cardiac failure and death. Recognition of risk factors (clinical and laboratory, including autoantibody profiles and tissue types) will help identify patients whose disease outcome may be improved with early and more aggressive treatment. The frequency of cancer has increased in scleroderma patients. Serious cardiac disease may be present in the absence of symptoms, whereas not all lung manifestations (reduced diffusion capacity) are necessarily progressive. More detailed descriptions of various manifestations and disease associations have been published. The development of noninvasive procedures will allow us to evaluate and follow up pulmonary and cardiac manifestations more objectively in such patients. The evolution of eosinophilic fasciitis into local scleroderma in children is of interest.
Current Opinion in Rheumatology 1993, 5:785-791