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Antisynthetase Syndrome — RACP Adult Medicine MCQ

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ModerateRheumatologyAntisynthetase SyndromeRACP Adult Medicine

A 50-year-old woman presents with proximal weakness, ILD (NSIP pattern on HRCT), mechanic hands (fissured hyperkeratosis of fingertips), arthritis, Raynaud phenomenon, and fever. CK is 3500 U/L. Anti-Jo-1 antibody is positive. What is the most likely inflammatory myopathy subtype?

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Correct answer: B — Antisynthetase syndrome

Myositis with ILD, mechanic hands, arthritis, Raynaud, and fever with anti-Jo-1 antibody defines antisynthetase syndrome. Anti-Jo-1 is the most common antisynthetase antibody (~20% of IIM). ILD (NSIP > OP pattern) is the major prognostic determinant. Treatment: corticosteroids + mycophenolate or azathioprine. Rituximab for refractory disease. ILD management drives therapy more than myositis.

Reference: ACR/EULAR – 2017 – IIM; Connors 2024 ASyS Review