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General Medicine · Rheumatology: antisynthetase syndrome
Sample itemA 45-year-old woman has fever, dry cough and breathlessness, with proximal muscle weakness, a raised CK, Raynaud phenomenon and symmetrical arthritis. Her fingers show rough, cracked skin along the lateral borders (mechanic's hands), and HRCT shows a basal non-specific interstitial pneumonia pattern. Which autoantibody is most likely positive?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Anti-Jo-1 (anti-histidyl tRNA synthetase)
Antisynthetase syndrome combines myositis, interstitial lung disease, arthritis, Raynaud phenomenon, fever and mechanic's hands, caused by antibodies to aminoacyl-tRNA synthetases, of which anti-Jo-1 is the most common. Lung involvement determines prognosis. Treatment is glucocorticoids with a steroid-sparing agent such as azathioprine or mycophenolate.
Option by option
A. Anti-Mi-2
Anti-Mi-2 is linked to classic dermatomyositis with a shawl sign and a good response to treatment.
B. Anti-centromere
Anti-centromere antibodies mark limited systemic sclerosis with CREST features.
C. Anti-signal recognition particle (anti-SRP)
Anti-SRP is associated with severe necrotising myopathy and cardiac involvement, with little lung disease.
D. Anti-Jo-1 (anti-histidyl tRNA synthetase) (correct)
Correct. Anti-Jo-1 is the most frequent antibody in this syndrome of myositis with interstitial lung disease.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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