Scleroderma renal crisis

Abstract

Systemic sclerosis (SSc) is a multisystem autoimmune rheumatic disease that results in substantial morbidity and significant mortality. Indeed, it is the autoimmune rheumatic disease with the highest case-specific mortality and it occurs as a result of organ-based complications. Historically, renal manifestations were a very important cause of death, but recent series show that most patients dying from SSc do so as a result of cardiopulmonary complications including, pulmonary hypertension, lung fibrosis, and myocardial involvement [1]. In many cases, there is co-occurrence of these different manifestations, and this may account for the particular severity of SSc. Although there are a range of ways in which the kidneys can be affected in SSc, the most significant renal manifestation is the scleroderma renal crisis (SRC), and this was historically a major cause of scleroderma-associated death. Other causes of renal pathology should however always be considered in SSc [2].