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抗MDA5抗体阳性的无皮肤表现的快速进展性间质性肺炎。

Anti-MDA5 antibody-positive rapidly progressive interstitial pneumonia without cutaneous manifestations.

作者信息

Aoyama Junichi, Hayashi Hiroki, Yajima Chika, Takoi Hiroyuki, Tanaka Toru, Kashiwada Takeru, Kokuho Nariaki, Terasaki Yasuhiro, Nishikawa Ayumi, Gono Takahisa, Kuwana Masataka, Saito Yoshinobu, Abe Shinji, Seike Masahiro, Gemma Akihiko

机构信息

Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan.

Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, Japan.

出版信息

Respir Med Case Rep. 2019 Jan 14;26:193-196. doi: 10.1016/j.rmcr.2019.01.012. eCollection 2019.

Abstract

A 47-year-old man was referred to our hospital with a 1-month history of fever and dyspnea after inhalation of insecticide in a confined space. We diagnosed rapidly progressive interstitial pneumonia. High-dose methylprednisolone, tacrolimus, and intermittent infusion of cyclophosphamide were administered. His condition rapidly deteriorated; therefore, extracorporeal membrane oxygenation therapy was performed. Unfortunately, he died 69 days after admission. Although typical skin findings suggestive of dermatomyositis were absent, anti-melanoma differentiation-associate gene (anti-MDA5) antibody was positive. Our findings suggest that in patients with hyperferritinemia and rapidly progressive interstitial lung disease (RP-ILD) demonstrating random ground glass shadows and peripheral consolidations by high-resolution computed tomography (HRCT) even if skin manifestations related to dermatomyositis are not complicated, we should assume anti-MDA5 antibody-positive interstitial pneumonia.

摘要

一名47岁男性因在密闭空间吸入杀虫剂后出现1个月的发热和呼吸困难症状被转诊至我院。我们诊断为快速进展性间质性肺炎。给予了大剂量甲泼尼龙、他克莫司以及间断静脉输注环磷酰胺治疗。他的病情迅速恶化,因此进行了体外膜肺氧合治疗。不幸的是,他在入院69天后死亡。尽管没有提示皮肌炎的典型皮肤表现,但抗黑色素瘤分化相关基因(anti-MDA5)抗体呈阳性。我们的研究结果表明,对于高铁蛋白血症和快速进展性间质性肺病(RP-ILD)患者,即使没有并发与皮肌炎相关的皮肤表现,若高分辨率计算机断层扫描(HRCT)显示有随机分布的磨玻璃影和外周实变,我们也应考虑抗MDA5抗体阳性的间质性肺炎。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d254/6350262/1afb9121c89e/gr1.jpg

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