呼吸臨床

【特集】日本人が見つけた自己抗原・抗原

企画：宮崎泰成


　すべての疾患において，原因が明らかにされると疾患概念は変ってくる。特に，免疫アレルギー疾患においては，その疾患の原因である自己抗原や抗原が明らかになると，病態が明らかになり，最終的にはそれまでの診断や治療戦略が見直され大きく変わることがある。

石坂公成博士のIgEの発見を始め，日本人はアレルギー性疾患の分野で多大な貢献をして来た。本企画においては，各専門家の皆様に，日本人による自己抗原・抗原の発見がその疾患概念や診断・治療にどのような与えた影響を与えたかについて概説していただく。

皮膚筋炎に伴う急速進行性間質性肺炎：MDA5

佐藤慎二*

*東海大学医学部内科学系リウマチ内科学（〒259-1193　神奈川県伊勢原市下糟屋143）


Rapidly progressive interstitial lung disease in patients with dermatomyositis: MDA5

Shinji Sato*
*Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Isehara


Keywords: 抗CADM-140抗体，抗MDA5抗体，皮膚筋炎，CADM，急速進行性間質性肺炎／anti-CADM-140 antibody，anti-MDA5 antibody，dermatomyositis，clinically amyopathic dermatomyositis (CADM), rapidly progressive interstitial lung disease（RP-ILD）, melanoma differentiation-associated gene 5(MDA5)


呼吸臨床 2017年1巻1号　論文No.e00013
Jpn Open J Respir Med　2017 Vol. 1 No. 1  Article No.e00013

DOI: 10.24557/kokyurinsho.1.e00013


掲載日：2017年10月2日


©️Shinji Sato. 本論文の複製権，翻訳権，上映権，譲渡権，貸与権，公衆送信権（送信可能化権を含む）は弊社に帰属し，それらの利用ならびに許諾等の管理は弊社が行います。


要旨

　抗MDA5抗体は，皮膚筋炎，特にそのサブタイプである典型的な皮膚筋炎の皮疹を呈しながら，臨床的に筋症状がまったくないかあるいはごく軽微な症例である無筋症性皮膚筋炎に見出されたDM特異自己抗体で，対応抗原はウイルス感染における自然免疫での感染防御機構で重要な役割を担っているmelanoma differentiation-associated gene 5（MDA5）である。同抗体陽性例は，臨床上，治療抵抗性･予後不良の急速進行性間質性肺炎を高頻度に併発するという特徴を有する。

文献

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  27. Sato S, et al. Anti-CADM-140/MDA5 autoantibody titer correlates with disease activity and predicts disease outcome in patients with dermatomyositis and rapidly progressive interstitial lung disease. Mod Rheumatol. 2013; 23: 496-502. 
  28. Matsushita T, et al. Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis. Br J Dermatol. 2017; 176: 395-402. 

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