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抗MDA5抗体阳性的临床无肌病性皮肌炎合并右侧单侧间质性肺疾病

Anti-MDA5 Antibody-positive Clinically Amyopathic Dermatomyositis Complicated by Unilateral Right-sided Interstitial Lung Disease.

作者信息

Kori Mayuko, Awano Nobuyasu, Inomata Minoru, Kuse Naoyuki, Tone Mari, Yoshimura Hanako, Jo Tatsunori, Takada Kohei, Kumasaka Toshio, Takemura Tamiko, Izumo Takehiro

机构信息

Department of Respiratory Medicine, Japanese Red Cross Medical Center, Japan.

Department of Pathology, Japanese Red Cross Medical Center, Japan.

出版信息

Intern Med. 2020;59(3):401-408. doi: 10.2169/internalmedicine.3604-19. Epub 2020 Feb 1.

Abstract

We herein report a case of anti-MDA5 antibody-positive, clinically amyopathic dermatomyositis complicated by unilateral interstitial lung disease (ILD) in a 78-year-old man with a history of left lung tumor resection. He was admitted due to a persistent fever and abnormal right pulmonary opacity. A transbronchial lung cryobiopsy revealed pulmonary fibrosis, and combined immunosuppressive therapy was initiated. Findings from multiple evaluations, including dynamic breathing magnetic resonance imaging, supported decreased perfusion, ventilation, and mobility of the left lung as etiological factors of unilateral lung ILD. When patients present with laterality of such findings, clinicians should be aware that atypical imaging findings may be observed.

摘要

我们在此报告一例78岁男性抗MDA5抗体阳性、临床无肌病性皮肌炎合并单侧间质性肺病(ILD)的病例,该患者有左肺肿瘤切除史。他因持续发热和右肺异常 opacity 入院。经支气管肺冷冻活检显示肺纤维化,并开始联合免疫抑制治疗。包括动态呼吸磁共振成像在内的多项评估结果支持左肺灌注、通气和活动度降低是单侧肺ILD的病因。当患者出现此类有侧别差异的表现时,临床医生应意识到可能会观察到非典型影像学表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/84f3/7028413/ff7e9f23dfc7/1349-7235-59-0401-g001.jpg

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