type:TOHO University Scholarly Publication

最終講義

我が国における特発性間質性肺炎(IIPs)研究の歴史は古く,40年間以上にわたり厚労省びまん性肺疾患に関する調査研究班が中心に症例を蓄積し詳細に分析してきた結果,日本から世界に発信された成果や見解が少しずつ国際ガイドラインに反映される時代となった.しかしながら,疾患多様性を有する特発性肺線維症(IPF)を単純かつ画一的にまとめ,しばしば経験する急性増悪のタイミングを的確に予測する指標を得ることは困難であり現在でも原因不明,予後不良の難治性疾患であることに変わりない.今後は,日本の国情に合ったエビデンスに基づいた標準的な治療法を意識した管理が必要不可欠であり,更なる個別化医療が要求されることになる.そのような中,我が国で初めてエビデンスに基づいたIPF治療ガイドライン2017が作成され,IPFの治療指針(慢性期における薬物・非薬物療法,急性増悪期,肺癌合併時)が明らかにされた.また,難病医療費助成制度の改訂により早期例に対しても医療費助成の範囲が拡大され,治療介入が可能となったことで更なる予後の改善が期待される.ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology that accounts for a large proportion of cases of idiopathic interstitial pneumonia. It has a very poor prognosis with a 5-year survival rate of 〓30%, and so far, there is no guideline in Japan offering an established effective therapy based on evidence. Recent years have seen many drugs being put forward as candidates in clinical trials for the assessment of their efficacy and safety. Back in the 2000s, a consensus statement on IPF treatment was formulated by bodies chiefly in Europe and North America. Subsequently a number of scientifically based randomized controlled trials (RCT) were planned and conducted, which resulted in the accumulation of evidence-based medicine (EBM) in literatures. It was on the basis of these results that the first EBM guideline was created in 2011. In Japan, the Manual for the Diagnosis and Treatment of Idiopathic Interstitial Pneumonia was issued in 2004 with the aim of establishing consistency with the European and North American consensus statement. The end of 2008 brought a world-first development with the marketing of the antifibrotic agent pirfenidone. To coincide with the 2011 issue of the EBM guideline, its content was incorporated in the Manual for the Diagnosis and Treatment of Idiopathic Interstitial Pneumonia (revised second edition) published in the same year. Between 2012 and the end of 2014, the results of further international clinical trials have been reported, leading to the revision of the European and North American EBM guideline on IPF treatment in 2015. In addition to the establishment of a basic therapy, there is also an urgent need to establish therapies to deal with complications because death occurs in many cases due to acute exacerbation or comorbid lung cancer. It was therefore decided to formulate the Japanese Guideline for Treatment of IPF 2017 in order to promote evidence-based clinical practice to further improve the quality of medical treatment in the clinical setting and to allow the benefits to be enjoyed by the public.