Japanese guidelines for the treatment of idiopathic pulmonary fibrosis 2023:Revised edition.

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor prognosis and an unknown cause that generally progresses to pulmonary fibrosis and leads to irreversible tissue alteration. The "Guidelines for the treatment of idiopathic pulmonary fibrosis 2017," specializing in the treatment of IPF for the first time in Japan and presenting evidence-based standard treatment methods suited to the state of affairs in Japan, was published in 2017, in line with the 2014 version of "Formulation procedure for Minds Clinical Practice Guidelines." Because new evidence had accumulated, we formulated the "Guidelines for the treatment of Idiopathic Pulmonary Fibrosis 2023 (revised 2nd edition)." While keeping the revision consistent with the ATS/ERS/JRS/ALAT IPF treatment guidelines, new clinical questions (CQs) on pulmonary hypertension were added to the chronic stage, in addition to acute exacerbation and comorbid lung cancer, which greatly affect the prognosis but are not described in the ATS/ERS/JRS/ALAT IPF guidelines. Regarding the advanced stages, we additionally created expert consensus-based advice for palliative care and lung transplantation. The number of CQs increased from 17 in the first edition to 24. It is important that these guidelines be used not only by respiratory specialists but also by general practitioners, patients, and their families; therefore, we plan to revise them appropriately in line with ever-advancing medical progress.
Competing Interests: Declaration of competing interest Masashi Bando received lecture fees from AstraZeneca K.K., Shionogi & Co., Ltd. and Nippon Boehringer Ingelheim Co., Ltd. Sakae Homma received lecture fees from Nippon Boehringer Ingelheim Co., Ltd. and belongs to Endowed departments by Shionogi & Co., Ltd., Chugai Pharmaceutical Co., Ltd., Teijin Pharma Ltd. and Nippon Boehringer Ingelheim Co., Ltd. Hiroshi Date received lecture fees from Covidien Japan Inc. and Johnson & Johnson K.K. and donations from Adachi Co., Ltd., Hogy Medical Co., Ltd. and Taiho Pharmaceutical Co., Ltd. Kazuma Kishi received lecture fees from AstraZeneca K.K., Ono Pharmaceutical Co., Ltd., Chugai Pharmaceutical Co., Ltd. and Nippon Boehringer Ingelheim Co., Ltd. and research funding from MSD K. K., Taiho Pharmaceutical Co., Ltd. and Chugai Pharmaceutical Co., Ltd. and donations from Chugai Pharmaceutical Co., Ltd., Eli Lilly Japan K.K. and Nippon Boehringer Ingelheim Co., Ltd. Atsushi Miyamoto received lecture fees from Nippon Boehringer Ingelheim Co., Ltd. Takeo Nakayama received lecture fees from Eli Lilly Japan K.K., Pfizer Japan, Inc. and Janssen Pharmaceutical K.K. and research finding from I&H Co., Ltd., Cocokarafine Group Co., Ltd. And Konica Minolta, Inc. and donations from Cancerscan Inc. and Yuyama Manufacturing Co., Ltd. Arata Azuma received lecture fees from Taiho Pharmaceutical Co., Ltd., Toray Industries, Inc. and Nippon Boehringer Ingelheim Co., Ltd. Yasuhiro Kondoh received lecture fees from Nippon Boehringer Ingelheim Co., Ltd., Bristol-Myers Squibb K.K. and Janssen Pharmaceutical K.K. Takeshi Johkoh received lecture fees from Nippon Boehringer Ingelheim Co., Ltd. Yasuhiko Nishioka received lecture fees from MSD K. K., AstraZeneca K.K., Chugai Pharmaceutical Co., Ltd. and Nippon Boehringer Ingelheim Co., Ltd. and research funding from Taiho Pharmaceutical Co., Ltd., TMS Co., Ltd., Chugai Pharmaceutical Co., Ltd. and Bonac Corporation and donations from Asahi Kasei Pharma Corporation, Ono Pharmaceutical Co., Ltd., Taiho Pharmaceutical Co., Ltd., Chugai Pharmaceutical Co., Ltd. and Eli Lilly Japan K.K. Junya Fukuoka received research funding from National Institute of Advanced Industrial Science and Technology and Future Corporation. Yasunari Miyazaki received donations from Chugai Pharmaceutical Co., Ltd. and Nippon Boehringer Ingelheim Co., Ltd. and lecture fees from AstraZeneca K.K. and Nippon Boehringer Ingelheim Co., Ltd. Ichiro Yoshino received lecture fees from MSD K. K., AstraZeneca K.K., Intuitive Surgical G.K., Ono Pharmaceutical Co., Ltd., Covidien Japan Inc., Johnson & Johnson K.K., Daiichi Sankyo Company, Ltd., Taiho Pharmaceutical Co., Ltd., Chugai Pharmaceutical Co. and Eli Lilly Japan K.K. and donations from Ono Pharmaceutical Co., Ltd., Taiho Pharmaceutical Co., Ltd., Eli Lilly Japan K.K. and Pfizer Japan, Inc. Takafumi Suda received lecture fees from AstraZeneca K.K. and Nippon Boehringer Ingelheim Co., Ltd. and research funding from Chugai Pharmaceutical Co., Nippon Boehringer Ingelheim Co. and Bristol-Myers Squibb K.K. and donations from Shionogi & Co., Ltd. and Chugai Pharmaceutical Co. Yoshihito Goto, Susumu Sakamoto and Hiroyoshi Yamauchi have no conflicts of interest.
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