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Anti-Synthetase Prognosis — SCE Rheumatology MCQ

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HardMyositisAnti-Synthetase PrognosisSCE Rheumatology

A patient with dermatomyositis has anti-MDA5 antibodies, modest creatine kinase, rapidly worsening hypoxaemia and new diffuse ground-glass change over two weeks. Infection sampling is underway. Which treatment principle best reflects BSR guidance once rapidly progressive ILD is judged likely?

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Correct answer: CUse high-dose glucocorticoid with cyclophosphamide or rituximab for immediate combination induction treatment

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

A is correct. Anti-MDA5 identifies a high-risk phenotype in which lung disease can progress catastrophically despite little muscle enzyme elevation. BSR guidance supports early aggressive induction for severe or rapidly progressive myositis-associated ILD, including glucocorticoids with cyclophosphamide or rituximab, while infection is assessed and managed in parallel. B confuses cutaneous treatment with organ-threatening disease. C imposes a dangerous wait for perfect diagnostic closure. D chooses a slower conventional DMARD with inadequate evidence for this emergency. E equates inflammatory ground-glass change with fixed fibrosis. Exact combination treatment is individualised in a specialist ILD/myositis service, often using additional agents, but delay based on the CK is the key avoidable error.

Reference: BSR guideline on management of paediatric, adolescent and adult idiopathic inflammatory myopathy: https://pmc.ncbi.nlm.nih.gov/articles/PMC9398208/