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Renal disease in systemic sclerosis (scleroderma), including scleroderma renal crisis

John Varga, MD
Andrew Z Fenves, MD
Section Editors
Richard J Glassock, MD, MACP
Gary C Curhan, MD, ScD
Deputy Editor
Alice M Sheridan, MD


The pathologic hallmarks of systemic sclerosis (SSc, scleroderma) are uncontrolled accumulation of collagen and widespread vascular lesions characterized by thickening of the vascular wall and narrowing of the vascular lumen. (See "Pathogenesis of systemic sclerosis (scleroderma)".)

Scleroderma may affect only the skin and subjacent tissues, or it may be associated with systemic involvement. The term systemic sclerosis is used when the characteristic skin lesions are associated with internal organ involvement. (See "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults".)

Renal involvement is common in patients with SSc, with the most serious manifestation being scleroderma renal crisis (SRC), which occurs in a minority of patients. (See 'Prevalence and manifestations of renal disease' below.)

SRC is characterized by three major features (see 'Clinical features' below):

Abrupt onset of moderate to severe hypertension that is typically associated with an increase in plasma renin activity

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Literature review current through: Nov 2017. | This topic last updated: Sep 25, 2017.
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