Immune Myopathy with Perimysial Pathology Associated with Interstitial Lung Disease and Anti-EJ Antibodies

Patrick M. Kwon, Lan Zhou, Rajeev Motiwala, Leslie D. Kerr, Susan C. Shin

Research output: Contribution to journalReview article

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.

Original languageEnglish (US)
Pages (from-to)223-227
Number of pages5
JournalJournal of Clinical Neuromuscular Disease
Volume18
Issue number4
DOIs
StatePublished - Jun 1 2017

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Amino Acyl-tRNA Synthetases
Interstitial Lung Diseases
Muscular Diseases
Anti-Idiotypic Antibodies
Pathology
Mycophenolic Acid
Myositis
Neural Conduction
Electromyography
Creatine Kinase
Deglutition Disorders
Prednisone
Fatigue
Blood Vessels
Weight Loss
Inflammation
Biopsy
Muscles
Therapeutics

Keywords

  • anti-EJ
  • anti-glycyl-tRNA
  • anti-jo-1 antibody
  • antisynthetase syndrome
  • immune myopathy with perimysial pathology
  • IMPP
  • interstitial lung disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Immune Myopathy with Perimysial Pathology Associated with Interstitial Lung Disease and Anti-EJ Antibodies. / Kwon, Patrick M.; Zhou, Lan; Motiwala, Rajeev; Kerr, Leslie D.; Shin, Susan C.

In: Journal of Clinical Neuromuscular Disease, Vol. 18, No. 4, 01.06.2017, p. 223-227.

Research output: Contribution to journalReview article

Kwon, Patrick M. ; Zhou, Lan ; Motiwala, Rajeev ; Kerr, Leslie D. ; Shin, Susan C. / Immune Myopathy with Perimysial Pathology Associated with Interstitial Lung Disease and Anti-EJ Antibodies. In: Journal of Clinical Neuromuscular Disease. 2017 ; Vol. 18, No. 4. pp. 223-227.
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