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Adult-onset Still disease — SCE Rheumatology MCQ

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HardPregnancy in rheumatic disease and rare conditionsAdult-onset Still diseaseSCE Rheumatology

A patient taking atorvastatin develops profound proximal weakness and CK 14,000 IU/L. Weakness and CK elevation persist eight weeks after statin withdrawal; anti-HMGCR antibodies are positive and biopsy shows necrosis with little inflammation. What is the best treatment principle?

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Reveal the answer and explanation

Correct answer: A — Immunosuppression plus early IVIG, CK and strength surveillance

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

B is correct. Anti-HMGCR immune-mediated necrotising myopathy persists after statin exposure ends and usually needs active immunotherapy; glucocorticoid-sparing treatment and early IVIG are commonly required, especially in severe weakness. A would re-expose the trigger and misunderstands persistence. C confuses toxic myalgia with antibody-mediated necrosis. D treats the wrong mechanism. E is not established monotherapy and makes antibody disappearance the target. Strength, swallowing, respiratory function and CK trend guide response, while future lipid management needs a non-statin cardiovascular strategy.

Reference: Nature Reviews Rheumatology: immune-mediated necrotising myopathy: https://www.nature.com/articles/s41584-020-00515-9