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Myositis-associated ILD — SCE Respiratory MCQ

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HardInterstitial lung diseaseMyositis-associated ILDSCE Respiratory

A 46-year-old woman has mechanic's hands, Raynaud phenomenon and proximal muscle aching. HRCT shows basilar ground-glass change with traction bronchiectasis; FVC is 62% predicted and CK is 1,100 IU/L. ANA is weakly positive, rheumatoid factor is negative and oxygen saturation falls to 86% on exertion. The ILD MDT suspects an inflammatory myopathy-associated ILD. What is the most appropriate investigation?

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Correct answer: B — Myositis-specific antibody panel including anti-Jo-1 and anti-MDA5

The clinical combination suggests antisynthetase or related myositis-associated ILD, so myositis-specific antibodies are the most appropriate targeted investigation. Alpha-1 antitrypsin deficiency and cystic fibrosis do not explain mechanic's hands and high CK. Identifying anti-MDA5 matters because it can be associated with rapidly progressive ILD.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/respiratory-conditions