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Autoinflammatory fever syndrome — SCE Rheumatology MCQ

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HardPaediatric RheumatologyAutoinflammatory fever syndromeSCE Rheumatology

A patient presented with renal-limited MPO-ANCA vasculitis and remains dialysis dependent after adequate induction, with no pulmonary, ENT, neurological or cutaneous activity. Repeated assessment finds no renal recovery. Which BSR principle is most relevant?

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Reveal the answer and explanation

Correct answer: A — Withdraw maintenance after confirming irreversible renal disease without extrarenal activity

Explanation lettering: C = shown as A · E = shown as B · D = shown as C · B = shown as D · A = shown as E

C is correct. BSR highlights a special risk–benefit exception: people with severe renal involvement who remain dialysis dependent have high infection risk, and those with renal-limited disease may not require ongoing immunotherapy once recovery is no longer expected and extrarenal activity is absent. Dialysis dependence alone is not proof of active inflammation; it may represent irreversible damage. ANCA negativity is not a treatment target, indefinite plasma exchange is unsupported, and dual maintenance magnifies toxicity. Continued surveillance remains necessary because extrarenal relapse can still occur.

Reference: BSR 2025 management recommendations for ANCA-associated vasculitis: https://pmc.ncbi.nlm.nih.gov/articles/PMC12316377/