A 52-year-old woman with diffuse cutaneous systemic sclerosis (dcSSc) develops acute hypertensive crisis (BP 220/130 mmHg) with microangiopathic haemolytic anaemia, thrombocytopenia, and rising creatinine. This is scleroderma renal crisis. The MOST important immediate treatment is:
- A Oral captopril (ACE inhibitor) as first-line, titrated rapidly ✓
- B IV hydralazine followed by oral nifedipine
- C Plasma exchange as the primary treatment modality
- D IV labetalol for BP control and dialysis preparation
Explanation
Scleroderma renal crisis is driven by endothelin-mediated vasospasm of renal afferent arterioles and activation of the renin-angiotensin system (not unlike renovascular hypertension). ACE inhibitors (especially captopril, due to rapid onset) are the cornerstone of treatment and have transformed the prognosis; they should be started urgently regardless of creatinine level. Unlike other hypertensive emergencies, continuation of ACEi even on dialysis has shown preserved partial renal recovery over months. Beta blockers worsen vasospasm via unopposed alpha-adrenergic activity. Plasma exchange has no established role. Nifedipine/hydralazine may be added as adjuncts.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.