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Systemic JIA — SCE Rheumatology MCQ

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HardPaediatric rheumatologySystemic JIASCE Rheumatology

A 9-year-old with juvenile dermatomyositis is anti-NXP2 positive and has persistent disease activity despite treatment. Which disease-related complication requires particularly close surveillance?

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Correct answer: D — Dystrophic soft-tissue calcinosis causing painful contractures

Anti-NXP2 positivity, younger onset, delayed treatment and prolonged active juvenile dermatomyositis are associated with calcinosis. Deposits may be painful, ulcerate or become infected, and can restrict movement, so assessment for calcinosis is specifically recommended while overall disease control is intensified.

Reference: BSR guideline for idiopathic inflammatory myopathy in children and adults (Published April 2022): https://academic.oup.com/rheumatology/article/61/5/1760/6555980; Calcinosis in juvenile dermatomyositis: updates on pathogenesis and treatment (Published March 2023): https://pmc.ncbi.nlm.nih.gov/articles/PMC10017873/