musculoskeletal and connective tissue disorders
Systemic Sclerosis
Prognosis for Systemic Sclerosis
Overall 10-year survival is 92% for limited systemic sclerosis and is 65% for diffuse systemic sclerosis. Predictors of early mortality include male sex, late onset, diffuse disease, pulmonary arterial hypertension, and renal crisis ( Prognosis reference ). The course depends on the type of disease (generalized vs limited) and antibody profiling but can be unpredictable. Patients with diffuse skin disease tend to have a more aggressive clinical course and eventually develop visceral complications (usually within the first 3 to 5 years), which, if severe, can lead to death. Heart failure may be intractable. Ventricular ectopy, even if asymptomatic, increases the risk of sudden death. Patients with limited systemic sclerosis (CREST syndrome) may have disease that is nonprogressive for long periods; visceral changes (eg, pulmonary hypertension caused by vascular disease of the lung, a peculiar form of biliary cirrhosis) eventually develop, but the course is often remarkably benign. 1.Hao Y, Hudson M, Baron M, et al: Early mortality in a multinational systemic sclerosis inception cohort.Arthritis Rheumatol69(5):1067–1077, 2017. doi: 10.1002/art.40027 