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

Respir Investig. 2024 May;62(3):402-418 doi: 10.1016/j.resinv.2024.02.014.
Abstract

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.

Metadata
Organisation: Bando, Masashi. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan. Electronic address: bando034@jichi.ac.jp. Homma, Sakae. Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan. Date, Hiroshi. Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan. Kishi, Kazuma. Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan. Yamauchi, Hiroyoshi. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan. Sakamoto, Susumu. Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan. Miyamoto, Atsushi. Department of Respiratory Medicine, Respiratory Center, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan. Goto, Yoshihito. Clinical Research Center, National Hospital Organization Kyoto Medical Center, 1-1, Mukaihata-cho, Fukakusa, Fushimi-ku, Kyoto, Kyoto, 612-8555, Japan. Nakayama, Takeo. Department of Health Informatics, Graduate School of Medicine and School of Public Health, Kyoto University, Yoshidakonoe-cho, Sakyo-ku, Kyoto, Kyoto, 606-8501, Japan. Azuma, Arata. Pulmonary Medicine, Tokorozawa Mihara General Hospital, 2-2934-3 Mihara-cho, Tokorozawa-shi, Saitama, 359-0045, Japan; Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan. Kondoh, Yasuhiro. Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, Aichi, 489-8642, Japan. Johkoh, Takeshi. Department of Radiology, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, Hyogo, 660-8511, Japan. Nishioka, Yasuhiko. Department of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan. Fukuoka, Junya. Department of Pathology Informatics, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan. Miyazaki, Yasunari. Department of Respiratory Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. Yoshino, Ichiro. Department of Thoracic Surgery, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita City, Chiba, 286-8520, Japan; Department of General Thoracic Surgery, Chiba University Hospital, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8677, Japan. Suda, Takafumi. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatus, 431-3192, Japan.
Funding: Non-industry funding
Publication type: Guideline
Organ: Lung
Language: English
MeSH terms: Humans; Japan; Idiopathic Pulmonary Fibrosis; Lung Diseases, Interstitial; Prognosis; Lung Neoplasms; Lung Transplantation