Chief causes of mortality in Systemic Sclerosis

What are the chief causes of mortality in Systemic Sclerosis?

With the introduction and success of ACE-inhibitor therapy in the management of SRC more than 30 years ago, the chief cause of Systemic Sclerosis-associated mortality has dramatically shifted from renal disease to lung and heart disease.

A recent study from the EUSTAR database (∼6000 Systemic Sclerosis patients) found that among Systemic Sclerosis-related deaths, 35% were attributed to ILD, 26% to PAH, 26% to cardiac causes (mainly heart failure and arrhythmias), and only 4% to renal disease.

The leading causes of non-Systemic Sclerosis-related death included infections (33%), malignancies (31%), and cardiovascular causes (29%).

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