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Antisynthetase syndrome with interstitial lung disease — ABIM Board MCQ

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HardPulmonary/Critical CareAntisynthetase syndrome with interstitial lung diseaseABIM Board

A 48-year-old woman has mechanic hands, Raynaud phenomenon, proximal muscle weakness, and dyspnea. CK is elevated. Chest CT shows basilar ground-glass opacities and traction bronchiectasis. Which of the following is the most likely diagnosis?

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Correct answer: B — Antisynthetase syndrome

Mechanic hands, Raynaud phenomenon, myositis, and interstitial lung disease suggest antisynthetase syndrome, commonly associated with anti-Jo-1 or other aminoacyl-tRNA synthetase antibodies.

Reference: ACR/Chest Interstitial Lung Disease in Systemic Autoimmune Rheumatic Diseases Guideline