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Scleroderma renal crisis — ESENeph MCQ

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HardGlomerulonephritisScleroderma renal crisisESENeph

A 52-year-old woman with diffuse cutaneous systemic sclerosis presents with headache and visual blurring. Blood pressure is 224/126 mmHg, creatinine has risen from 82 to 238 micromol/L and urinalysis shows protein 1+. Platelets are 94 x 10^9/L and blood film shows fragments. What is the most appropriate management?

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Correct answer: E — Start an ACE inhibitor immediately and titrate rapidly

This is scleroderma renal crisis with malignant hypertension, AKI and thrombotic microangiopathy; ACE inhibition is the key disease-modifying treatment even when creatinine is elevated. High-dose corticosteroids may precipitate or worsen scleroderma renal crisis. Plasma exchange is not the primary treatment unless another TMA diagnosis becomes likely. The pearl is that prompt ACE inhibitor therapy can improve survival, and dialysis may be temporary in some patients.

Reference: BSR systemic sclerosis guidance; KDIGO blood pressure guidance