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Myositis associated ILD — SCE Rheumatology MCQ

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HardConnective Tissue DiseasesMyositis associated ILDSCE Rheumatology

A 48-year-old woman has rapidly progressive dyspnoea, fever, mechanic's hands and mild proximal weakness. CK is 520 IU/L. ANA is positive and anti-MDA5 antibody is detected. HRCT shows diffuse ground-glass opacity and early organising pneumonia. Infection screen is negative. What is the most important complication to screen for?

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

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Correct answer: B — Rapidly progressive interstitial lung disease

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

Rapidly progressive interstitial lung disease is best because anti-MDA5 dermatomyositis is strongly associated with rapidly progressive ILD, sometimes with modest CK elevation. B is less suitable because tophaceous gout does not cause this antibody-lung phenotype; C is less suitable because calcific uraemic arteriolopathy occurs in advanced kidney disease; D is less suitable because atlantoaxial subluxation is an RA complication; E is less suitable because renal crisis relates to systemic sclerosis and severe hypertension. Clinical pearl: myositis antibody profiles can be more prognostically important than CK level.

Reference: BSR idiopathic inflammatory myopathy guideline 2022; ERS/EULAR CTD-ILD guidance