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Scleroderma Renal Crisis ACEi — ESENeph MCQ

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ModerateAcute Kidney InjuryScleroderma Renal Crisis ACEiESENeph

A 35-year-old man presents with AKI in the context of scleroderma. He has rapidly rising creatinine (100 to 450 umol/L in 5 days), severe hypertension (BP 220/130 mmHg), and microangiopathic haemolytic anaemia. What is the diagnosis and key treatment?

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Correct answer: C — Scleroderma renal crisis (SRC) – ACE inhibitor is the specific first-line treatment

Scleroderma renal crisis (SRC) presents with acute onset severe hypertension, rapidly progressive AKI, and TMA. ACE inhibitors are the specific first-line treatment – they block the RAAS activation that drives the pathology (renin-mediated vasoconstriction of renal arterioles). Captopril (short-acting, titratable) is traditionally preferred. Despite previous concerns, ACEi should be started even if the patient is normotensive or has declining GFR. Approximately 50% of SRC patients require temporary dialysis, but many recover kidney function with ACEi. Prior glucocorticoid exposure (>15 mg Prednisolone) is a risk factor for SRC.

Reference: KDIGO 2021 – GN Guideline; Steen et al 1990 – ACEi in SRC