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Antisynthetase syndrome — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologyAntisynthetase syndromeABIM Board

A 46-year-old has inflammatory arthritis, mechanic hands, Raynaud phenomenon, proximal weakness and progressive interstitial lung disease. CK is elevated and anti-Jo-1 antibody is present. What is the most likely syndrome?

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Correct answer: B — Antisynthetase syndrome with anti-Jo-1 antibody

The best answer is “Antisynthetase syndrome with anti-Jo-1 antibody”. An aminoacyl-tRNA synthetase antibody such as anti-Jo-1, together with myositis, inflammatory arthritis, mechanic hands and ILD, defines the antisynthetase phenotype. Raynaud phenomenon is common. The alternatives do not explain this antibody and organ cluster. Interstitial lung disease may dominate morbidity and requires coordinated pulmonary assessment rather than being treated as an incidental antibody finding.

Reference: 2023 ACR/CHEST Guideline for Interstitial Lung Disease in Systemic Autoimmune Rheumatic Diseases: https://rheumatology.org/interstitial-lung-disease-guideline