skip to main content

Enthesitis-related arthritis — SCE Rheumatology MCQ

Instant feedback + full explanation. One question, done properly.

HardPaediatric RheumatologyEnthesitis-related arthritisSCE Rheumatology

A 16-year-old with systemic JIA develops encephalopathy, hepatomegaly and coagulopathy. Ferritin has risen from 4,000 to 48,000 micrograms/L while platelets, ESR and fibrinogen are falling; AST and triglycerides are rising. Cultures are pending and marrow has not been sampled. What is the best immediate strategy?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — Start high-dose glucocorticoid plus early anakinra, adding ciclosporin after specialist assessment

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

C is correct. The discordant fall in ESR, platelets and fibrinogen with rapidly rising ferritin, AST, triglycerides and neurological dysfunction is classic evolving MAS. EULAR/PReS supports urgent high-dose glucocorticoid with early cytokine-directed treatment, commonly anakinra, and ciclosporin in selected severe disease; marrow haemophagocytosis is neither required nor sufficiently sensitive. A delays lifesaving therapy. B imports an obligatory primary-HLH pathway. D mistakes consumption for recovery. E is inadequate monotherapy in fulminant MAS and IL-6 blockade can obscure CRP.

Reference: EULAR/PReS recommendations for Still disease 2024: https://ard.bmj.com/content/83/12/1614