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Systemic juvenile idiopathic arthritis with MAS — SCE Rheumatology MCQ

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HardPaediatric rheumatologySystemic juvenile idiopathic arthritis with MASSCE Rheumatology

A patient with Still disease develops persistent fever, encephalopathy, falling platelets, hypofibrinogenaemia, hepatitis and rapidly rising ferritin. Cultures are pending. What is the most appropriate immediate immunomodulatory framework?

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Correct answer: B — Pulse methylprednisolone and add anakinra or ciclosporin while treating triggers

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

E is correct. MAS is a time-critical emergency: high-dose glucocorticoids are foundational, with anakinra, ciclosporin or selected interferon-gamma inhibition considered early according to phenotype and response. Infection investigation and treatment proceed in parallel. A and C delay life-saving therapy. B under-treats fulminant disease. D imports a universal primary-HLH strategy into secondary MAS.

Reference: EULAR/PReS recommendations for diagnosis and management of Still disease: https://pmc.ncbi.nlm.nih.gov/articles/PMC11672000/