Introduction
Antisynthetase syndrome is a constellation of “mechanic’s hands,”
interstitial lung disease (70%), myositis, arthritis (50%), rash
(30%), sicca syndrome, Raynaud’s phenomenon and constitutional
symptoms.1-3 Antibodies are produced to aminoacyl tRNA
synthetases that catalyze attachment of amino acids to tRNA and recruit
inflammatory cells to sites of muscle and lung
injury.1 In EJ-antibody antisynthetase syndrome, the
antigen is glycyl tRNA synthetase (GARS).2
Eight anti-aminoacyl-tRNA synthetases (ARS) have been described; anti-EJ
comprise 2-5%.3 Myositis is a late manifestation at
17 months.2 EMG, muscle MRI and biopsy show
steroid-responsive myopathy.3 Long term
immunosuppression is required 80% of the time.4