" /> 【特集】特発性間質性肺炎の診療の現状と将来展望■膠原病の匂いのする間質性肺疾患：IPAFを中心に■穗積宏尚，ほか |
呼吸臨床

【特集】特発性間質性肺炎の診療の現状と将来展望

企画：小倉高志


　最近，特発性間質性肺炎(IIPs)は次の点で注目されています。①IIPsの中でも頻度の高く，難治性といわれている特発性肺線維症（IPF）に対する２つの抗線維化薬（ピルフェニドン，ニンテダティブ）が出現して，IIPsならIPFか，それ以外のnon-IPFかの鑑別がすることが治療選択や予後に大きく影響を与えるようになりました。②無症状で潜在性な間質性肺炎患者が増加している可能性が示唆されています。すなわち，いくつもの成人病のコホート，肺癌やCOPDのCT検診でInterstitial Lung Abnormalities(ILA)を８〜10％に認めて，その一部はIPFに進展する可能性あるという報告がされています。呼吸器内科医すべてが，IIPsの診断，治療にかかわる機会が多くなることが予想されます。今回，IIPs診療の第一線で治療にあたっている専門家にIIPsの最近の診断と治療について解説していただきます。

膠原病の匂いのする間質性肺疾患：IPAFを中心に

穗積宏尚*，須田隆文*

*浜松医科大学内科学第二講座（〒431-3192 静岡県浜松市東区半田山1-20-1）


Interstitial lung disease with a rheumatic flavor: a focus on IPAF

Hironao Hozumi*，Takafumi Suda*

*Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu


Keywords：特発性間質性肺炎，IPAF，自己免疫性，間質性肺疾患，膠原病／idiopathic interstitial pneumonias，interstitial pneumonia with autoimmune features，autoimmune，interstitial lung disease，connective tissue disease


呼吸臨床 2018年2巻8号　論文No.e00040
Jpn Open J Respir Med 2018 Vol.2 No.8  Article No.e00040

DOI: 10.24557/kokyurinsho.2.e00040


掲載日：2018年8月17日


©️Hironao Hozumi, et al. 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

　特発性間質性肺炎の中には，膠原病らしい所見をもちながらも確立した診断基準を満たさない「膠原病の匂いのする間質性肺疾患」が存在する。このような一群を分類するための基準は混沌としていたが，近年，interstitial pneumonia with autoimmune features（IPAF）という概念に統一されようとしている。本稿では，この「膠原病の匂いのする間質性肺疾患」がIPAFという概念に至るまでの経緯と，最近報告されたIPAFに関する研究について概説する。


文献

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