A 35-year-old woman with diffuse cutaneous systemic sclerosis develops acute kidney injury with blood pressure 180/110 mmHg. Urinalysis shows mild proteinuria and granular casts. What is the BEST initial treatment for scleroderma renal crisis?
- A Oral prednisolone 1 mg/kg/day
- B Captopril 6.25 mg three times daily, titrated rapidly ✓
- C IV methylprednisolone pulse therapy
- D Mycophenolate mofetil with amlodipine
Explanation
Scleroderma renal crisis (SRC) is a medical emergency characterized by accelerated hypertension, AKI, and microangiopathic hemolytic anemia in diffuse SSc, associated with anti-RNA polymerase III antibodies. ACE inhibitors, particularly captopril, are the cornerstone treatment—they reverse the renal vasospasm and angiotensin II–mediated intrarenal hypertension that drives cortical ischemia. High-dose corticosteroids are a risk factor for precipitating SRC, not a treatment. Prompt and aggressive ACE inhibitor titration is life-saving; dialysis may be required but renal recovery can occur months later.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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