Systemic sclerosis is a rare autoimmune disorder with a historically bad prognosis. Survival has been improving over time and we can currently estimate a 1-year survival, 94.9; a 5-year survival, 84.4; a 10-year survival, 70.9 and a 20-year survival, 44.9%, from the time of diagnosis. Accordingly, mortality has been decreasing over time, being the overall standardized mortality ratio (SMR) 2.72 (1.90–3.83), SMR 2.4 after 1990. Among the SSc-related causes of death, the lung death is the most important cause and its relative percentage is increasing over time since the introduction of ACE inhibitors for the treatment of scleroderma renal crisis (SRC) in early 1990s. Among the SSc-non-related causes of death, cancer, infection and cardiovascular disorders are the leading causes of death. Risk factor predictors of poor outcomes are an elder age at diagnosis, the male gender, diffuse subset, visceral involvement and non-Raynaud’s phenomenon onset.
Part of the book: Systemic Sclerosis