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在抗黑色素瘤分化相关基因5抗体阳性的临床无肌病性皮肌炎中,典型皮肌炎症状出现之前的缓慢进展性间质性肺疾病。

Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis.

作者信息

Isono Taisuke, Nakajima Hiromi, Takano Kenji, Kobayashi Yoichi, Kawabata Yoshinori, Shimizu Yoshihiko, Takayanagi Noboru

机构信息

Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan.

Departments of Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan.

出版信息

Respir Med Case Rep. 2021 Jul 27;34:101491. doi: 10.1016/j.rmcr.2021.101491. eCollection 2021.

DOI:10.1016/j.rmcr.2021.101491
PMID:34381685
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8339249/
Abstract

A 73-year-old woman who visited our hospital complaining of dry cough for three months was refractory to antimicrobial therapy. Chest computed tomography revealed subpleural consolidation. Specimens obtained from surgical lung biopsy revealed subpleural perilobular airspace organization and fibrosis. After the biopsy, mechanic's hand and Gottron's papules appeared, and anti-melanoma differentiation-associated gene 5 (MDA5) antibody was found to be positive. Subsequently, anti-MDA5 antibody measured in cryopreserved serum from her first admission proved to be positive. It is difficult to suspect the presence of anti-MDA-5 antibody in patients with interstitial lung disease without typical dermatomyositis symptoms or slow disease progression.

摘要

一名73岁女性因干咳3个月前来我院就诊,抗菌治疗无效。胸部计算机断层扫描显示胸膜下实变。手术肺活检获取的标本显示胸膜下小叶周围气腔组织化和纤维化。活检后,出现技工手和Gottron丘疹,且抗黑色素瘤分化相关基因5(MDA5)抗体检测呈阳性。随后,对其首次入院时冻存血清检测发现抗MDA5抗体也呈阳性。对于没有典型皮肌炎症状或疾病进展缓慢的间质性肺病患者,很难怀疑其存在抗MDA-5抗体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/dfec2d21e597/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/7f8d4cede5c8/gr1.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/d0a8a63a7a34/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/72dde27b4d07/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/dfec2d21e597/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/7f8d4cede5c8/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/54853cc15794/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/58b1770e8e5f/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/d0a8a63a7a34/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/72dde27b4d07/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbe0/8339249/dfec2d21e597/gr6.jpg

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Front Med (Lausanne). 2020 Mar 10;7:77. doi: 10.3389/fmed.2020.00077. eCollection 2020.
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Fatal and extensive multiorgan hemorrhages in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: An autopsy case report.抗黑色素瘤分化相关基因5抗体阳性皮肌炎中的致死性广泛性多器官出血:一例尸检病例报告
Medicine (Baltimore). 2020 Jan;99(3):e18600. doi: 10.1097/MD.0000000000018600.
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Respir Med Case Rep. 2024 Jun 18;51:102072. doi: 10.1016/j.rmcr.2024.102072. eCollection 2024.
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