A 42-year-old woman with systemic sclerosis (diffuse cutaneous form) has a serum creatinine that has doubled over 5 days. Blood pressure is 175/108 mmHg. Urinalysis shows mild proteinuria. Peripheral smear shows schistocytes. What is the most appropriate immediate therapy?
- A ACE inhibitor (captopril) initiated urgently ✓
- B High-dose corticosteroids (prednisolone 1 mg/kg/day)
- C Cyclophosphamide IV pulse
- D Plasma exchange followed by rituximab
Explanation
This clinical picture is scleroderma renal crisis (SRC) — an emergency characterised by abrupt onset of hypertension, microangiopathic haemolytic anaemia (schistocytes), and rapidly progressive renal insufficiency in diffuse systemic sclerosis. ACE inhibitors, specifically captopril, are the cornerstone of treatment and have transformed the prognosis of SRC — they must be started urgently even if the patient requires dialysis subsequently. Corticosteroids are actually a risk factor for precipitating SRC and are contraindicated. Cyclophosphamide and plasma exchange are not the standard of care for SRC.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.