伴有肌束膜病理改变、间质性肺疾病及抗EJ抗体的免疫性肌病

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

作者信息

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

机构信息

*Neuromuscular Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY; and †Division of Rheumatology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY.

出版信息

J Clin Neuromuscul Dis. 2017 Jun;18(4):223-227. doi: 10.1097/CND.0000000000000148.

DOI:10.1097/CND.0000000000000148

PMID:28538253

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.

摘要

目的

我们报告一例伴有肌束膜病理改变的免疫性肌病病例，该病例与抗甘氨酰 - 转移RNA合成酶（抗EJ）抗体相关，且治疗反应良好。

方法

病历回顾。

结果

一名36岁女性，出现3个月的疲劳、体重减轻、肩胛腓骨肌分布区进行性无力及吞咽困难。神经传导研究、肌电图和超声提示为激惹性肌病。她的肌酸激酶显著升高，抗甘氨酰 - 转移RNA合成酶（抗EJ）抗体呈阳性。左侧肱二头肌活检显示肌束膜及周围肌束膜血管炎症，肌束膜有局灶性断裂。进一步评估发现间质性肺病。泼尼松和霉酚酸酯治疗使她的症状有显著临床改善。