A patient with scleroderma (systemic sclerosis) develops acute-onset oliguria, hypertensive emergency (BP 220/140 mmHg), and microangiopathic haemolytic anaemia. Creatinine rises from 0.9 to 3.8 mg/dL over 3 days. What is the most important specific treatment for scleroderma renal crisis?
- A Captopril or another ACE inhibitor started immediately regardless of creatinine ✓
- B Emergency haemodialysis with ACE inhibitor avoidance to preserve residual renal function
- C Corticosteroids at high dose to suppress immune-mediated vasculopathy
- D Plasmapheresis to remove endothelin and anti-endothelial antibodies
Explanation
Scleroderma renal crisis (SRC) is an emergency requiring immediate ACE inhibitor therapy — captopril is the drug of choice because it blocks the renin-angiotensin activation central to SRC pathophysiology. ACE inhibitors should NOT be withheld even if creatinine is rising, as they reduce mortality, aid renal recovery, and some patients recover enough renal function to discontinue dialysis months later. High-dose corticosteroids are contraindicated as they precipitate SRC. Prior corticosteroid use (>15 mg/day prednisolone) is the strongest risk factor for SRC development.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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