Immune Myopathy With Perimysial Pathology Associated With Interstitial Lung Disease and Anti-EJ Antibodies. Issue 4 (June 2017)

• Record Type: Journal Article
• Authors: Kwon, Patrick M.; Zhou, Lan; Motiwala, Rajeev; Kerr, Leslie D.; Shin, Susan C.
• Is Part Of: Journal of clinical neuromuscular disease. Volume 18:Issue 4(2017:Jun.)
• Publication Date: 2017-06
• Subjects: anti-glycyl-tRNA -- anti-EJ -- IMPP -- immune myopathy with perimysial pathology -- antisynthetase syndrome -- interstitial lung disease -- anti-jo-1 antibody; Neuromuscular diseases -- Periodicals; 616.744005
• DOI: 10.1097/CND.0000000000000148
• Languages: English
• ISSNs: 1522-0443

Abstract

Abstract: Objectives: We report a case of immune myopathy with perimysial pathology associated with anti-glycyl-transfer RNA synthetase (anti-EJ) antibody and an excellent treatment response. Methods: Chart review. Results: A 36-year-old woman presented with 3 months of fatigue, weight loss, progressive weakness in a scapuloperoneal distribution, and dysphagia. Nerve conduction studies, electromyography, and ultrasound suggested an irritable myopathy. She had marked elevations of creatine kinase and positive anti-glycyl-transfer RNA synthetase (anti-EJ) antibodies. A left biceps muscle biopsy revealed inflammation of the perimysium and surrounding perimysial blood vessels with focal fragmentation of the perimysium. Further evaluation revealed interstitial lung disease. Treatment with prednisone and mycophenolate mofetil led to marked clinical improvement of her symptoms. Conclusions: Our case adds to the growing spectrum of inflammatory myopathies and highlights the importance of performing a comprehensive, multisystem workup.