Anti-Synthetase Syndrome — SCE Rheumatology MCQ

Instant feedback + full explanation. One question, done properly.

ModerateAutoimmune SerologyAnti-Synthetase SyndromeSCE Rheumatology

A 48-year-old woman with proximal muscle weakness and an elevated creatine kinase is positive for anti-Jo-1 antibodies. She develops progressive dyspnoea and hyperkeratotic fissuring along the radial aspects of her fingers. HRCT shows lower-lobe-predominant ground-glass opacities with an organising pneumonia pattern. Which diagnosis best unifies these findings?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: D — Anti-synthetase syndrome

Anti-synthetase syndrome is the unifying diagnosis. Anti-Jo-1 targets histidyl-tRNA synthetase and, together with inflammatory myopathy, interstitial lung disease and mechanic's hands, forms the characteristic phenotype. Lower-lobe ground-glass change with an organising pneumonia or NSIP pattern is compatible with its pulmonary involvement. Anti-MDA5 dermatomyositis is associated particularly with cutaneous dermatomyositis and rapidly progressive ILD, rather than anti-Jo-1 positivity. Scleroderma–myositis overlap requires systemic-sclerosis features or associated antibodies. Immune-mediated necrotising myopathy is more closely associated with anti-SRP or anti-HMGCR antibodies and does not explain the mechanic's hands and ILD. Mixed connective tissue disease requires anti-U1-RNP antibodies and an appropriate overlap phenotype.

Reference: Barratt SL, Adamali HH, Cotton C, et al. Clinicoserological features of antisynthetase syndrome-associated interstitial lung disease presenting to respiratory services: Introduction. BMJ Open Respiratory Research. 2021;8:e000829. https://bmjopenrespres.bmj.com/content/8/1/e000829