A 48-year-old woman has diffuse cutaneous systemic sclerosis (dcSSc) complicated by scleroderma renal crisis. Her BP is 195/110 mmHg and serum creatinine has risen from 0.9 to 3.2 mg/dL over 5 days. Anti-RNA polymerase III antibodies are positive. The treatment of CHOICE is:
- A High-dose corticosteroids (prednisolone 60 mg/day)
- B Calcium channel blocker (amlodipine)
- C ARB (losartan) combined with amlodipine
- D ACE inhibitor (captopril) ✓
Explanation
Scleroderma renal crisis (SRC) is a hypertensive emergency driven by renal angiotensin system hyperactivation due to intimal proliferation. ACE inhibitors (particularly captopril, which has rapid onset and can be titrated) are the ONLY treatment shown to improve renal and patient survival in SRC. Anti-RNA polymerase III antibodies are strongly associated with SRC. Corticosteroids (doses ≥15 mg/day) are a known precipitant of SRC and are contraindicated. ARBs have not been shown to be equivalent. Early and aggressive ACE inhibitor therapy, even in dialysis-dependent patients, may allow renal recovery.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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