Scleroderma renal crisis

Scleroderma renal crisis (SRC) occurs abruptly in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in the disease. It is characterized by malignant hypertension, hyperreninomia, azotemia, microangiopathic hemolytic anemia, and renal failure. Angiotensin converting enzyme (ACE) inhibitors have dramatically changed the course of this almost uniformly fatal complication. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome. Chronic nonrenal crisis renal insufficiency is unusual and rarely progresses to significant renal dysfunction.