Anti-Synthetase Syndrome — SCE Rheumatology MCQ

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ModerateMyositisAnti-Synthetase SyndromeSCE Rheumatology

A 45-year-old woman with dermatomyositis develops inflammatory arthritis, interstitial lung disease and hyperkeratotic fissuring along the radial aspects of her fingers consistent with mechanic's hands. Testing for myositis-specific antibodies is positive for anti-PL-7. Which syndrome best unifies these findings?

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Correct answer: D — Antisynthetase syndrome

Anti-PL-7 is directed against threonyl-tRNA synthetase and is one of the aminoacyl-tRNA synthetase antibodies defining antisynthetase syndrome. The characteristic clinical spectrum comprises inflammatory myopathy, interstitial lung disease and inflammatory arthritis, with mechanic's hands, Raynaud phenomenon and fever as additional features. Anti-PL-7 disease is particularly associated with prominent or severe lung involvement. Dermatomyositis morphology may occur within the antisynthetase spectrum, so option E does not adequately unify the antibody and systemic phenotype. Immune-mediated necrotising myopathy is more typically associated with anti-SRP or anti-HMGCR antibodies and does not explain mechanic's hands or arthritis. Inclusion body myositis has a different weakness pattern and demographic profile. Systemic sclerosis overlap would require supporting scleroderma features or relevant overlap antibodies.

Reference: Abel A et al. Phenotypic Profiles Among 72 Caucasian and Afro-Caribbean Patients with Antisynthetase Syndrome Involving Anti-PL7 or Anti-PL12 Autoantibodies. European Journal of Internal Medicine. 2023;115:104–113. https://pubmed.ncbi.nlm.nih.gov/37330316/