Homma Sakae, Bando Masashi, Azuma Arata, Sakamoto Susumu, Sugino Keishi, Ishii Yoshiki, Izumi Shinyu, Inase Naohiko, Inoue Yoshikazu, Ebina Masahito, Ogura Takashi, Kishi Kazuma, Kishaba Tomoo, Kido Takashi, Gemma Akihiko, Goto Yoshihito, Sasaki Shinichi, Johkoh Takeshi, Suda Takafumi, Takahashi Kazuhisa, Takahashi Hiroki, Taguchi Yoshio, Date Hiroshi, Taniguchi Hiroyuki, Nakayama Takeo, Nishioka Yasuhiko, Hasegawa Yoshinori, Hattori Noboru, Fukuoka Junya, Miyamoto Atsushi, Mukae Hiroshi, Yokoyama Akihito, Yoshino Ichiro, Watanabe Kentaro
Toho University, Faculty of Medicine, Omori Medical Center, Department of Respiratory Medicine, 6-11-1 Omori Nishi, Ota-ku, Tokyo 143-8541, Japan.
Jichi Medical University, Department of Medicine, Division of Pulmonary Medicine, Japan.
Respir Investig. 2018 Jul;56(4):268-291. doi: 10.1016/j.resinv.2018.03.003. Epub 2018 Jul 3.
Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology which 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% or below, and so far there has been no guideline in Japan offering an established effective therapy based on evidence. In addition to the establishment of basic therapies, there is also an urgent need to establish therapies to deal with complications, as death occurs in many cases due to acute exacerbation or comorbid lung cancer. It was therefore decided to formulate a guideline 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.
特发性肺纤维化（IPF）是一种病因不明的疾病，在特发性间质性肺炎病例中占很大比例。其预后很差，5年生存率为30%或更低，而且迄今为止在日本尚无基于证据的既定有效治疗指南。除了确立基础治疗方法外，还迫切需要确立应对并发症的治疗方法，因为许多病例死于急性加重或合并肺癌。因此，决定制定一项指南，以促进基于证据的临床实践，进一步提高临床环境中的医疗质量，并让公众受益。
