A woman with diffuse cutaneous systemic sclerosis develops worsening hypertension (BP 190/110), microangiopathic haemolytic anaemia, and creatinine rising to 3.2 mg/dL over days. What is the cornerstone treatment that transformed prognosis of this complication?
- A ACE inhibitors (captopril) ✓
- B High-dose corticosteroids
- C Plasmapheresis
- D IV cyclophosphamide
Correct answer: A. ACE inhibitors (captopril)
Explanation
Scleroderma renal crisis (SRC) is a life-threatening complication of diffuse SSc, characterised by accelerated hypertension and thrombotic microangiopathy. ACE inhibitors (especially captopril, initiated immediately and aggressively titrated) are the cornerstone treatment — they dramatically improved survival from <10% to >65% at 1 year. Corticosteroids are contraindicated as they are a major precipitant of SRC. Anti-RNA polymerase III antibodies identify highest-risk patients. Blood pressure control with ACEi is maintained even in dialysis-dependent patients.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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