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Scleroderma renal crisis — SCE Acute Medicine MCQ

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HardMusculoskeletal SystemScleroderma renal crisisSCE Acute Medicine

A 49-year-old woman presents with acute shortness of breath and severe hypertension. She has diffuse skin thickening, Raynaud's phenomenon and new microangiopathic haemolytic anaemia. Creatinine has risen from 72 to 246 micromol/L in three weeks and urine dip shows protein 2+. What is the most likely diagnosis?

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Correct answer: C — Scleroderma renal crisis

Rapid renal failure, severe hypertension and microangiopathic haemolysis in systemic sclerosis indicate scleroderma renal crisis. ANCA vasculitis may cause pulmonary-renal syndrome but the skin and Raynaud's phenotype with malignant hypertension strongly favour scleroderma renal crisis. ACE inhibitor therapy is central even if creatinine rises initially. The clinical trap is to treat it as generic hypertensive nephropathy rather than disease-specific emergency.

Reference: BSR systemic sclerosis guideline, BNF