Scleroderma renal crisis

Renal crisis occurs in patients who have systemic sclerosis with rapidly progressive diffuse cutaneous thickening early in their disease. Scleroderma renal crisis is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. This complication was almost uniformly fatal, but in most cases it can now be successfully treated with angiotensin-converting enzyme (ACE) inhibitors. This therapy has improved survival, reduced the requirement for dialysis, and often allowed for the discontinuation of dialysis 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome.