Japanese guideline for the treatment of idiopathic pulmonary fibrosis

Sakae Homma; Masashi Bando; Arata Azuma; Susumu Sakamoto; Keishi Sugino; Yoshiki Ishii; Shinyu Izumi; Naohiko Inase; Yoshikazu Inoue; Masahito Ebina; Takashi Ogura; Kazuma Kishi; Tomoo Kishaba; Takashi Kido; Akihiko Gemma; Yoshihito Goto; Shinichi Sasaki; Takeshi Johkoh; Takafumi Suda; Kazuhisa Takahashi; Hiroki Takahashi; Yoshio Taguchi; Hiroshi Date; Hiroyuki Taniguchi; Takeo Nakayama; Yasuhiko Nishioka; Yoshinori Hasegawa; Noboru Hattori; Junya Fukuoka; Atsushi Miyamoto; Hiroshi Mukae; Akihito Yokoyama; Ichiro Yoshino; Kentaro Watanabe; Ministry of Health, Labour and Welfare, the Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases, and Japanese Respiratory Society

doi:10.1016/j.resinv.2018.03.003

Japanese guideline for the treatment of idiopathic pulmonary fibrosis

Respir Investig. 2018 Jul;56(4):268-291. doi: 10.1016/j.resinv.2018.03.003. Epub 2018 Jul 3.

Authors

Sakae Homma¹, Masashi Bando², Arata Azuma³, Susumu Sakamoto⁴, Keishi Sugino⁴, Yoshiki Ishii⁵, Shinyu Izumi⁶, Naohiko Inase⁷, Yoshikazu Inoue⁸, Masahito Ebina⁹, Takashi Ogura¹⁰, Kazuma Kishi¹¹, Tomoo Kishaba¹², Takashi Kido¹³, Akihiko Gemma³, Yoshihito Goto¹⁴, Shinichi Sasaki¹⁵, Takeshi Johkoh¹⁶, Takafumi Suda¹⁷, Kazuhisa Takahashi¹⁸, Hiroki Takahashi¹⁹, Yoshio Taguchi²⁰, Hiroshi Date²¹, Hiroyuki Taniguchi²², Takeo Nakayama¹⁴, Yasuhiko Nishioka²³, Yoshinori Hasegawa²⁴, Noboru Hattori²⁵, Junya Fukuoka²⁶, Atsushi Miyamoto¹¹, Hiroshi Mukae²⁷, Akihito Yokoyama²⁸, Ichiro Yoshino²⁹, Kentaro Watanabe³⁰; Ministry of Health, Labour and Welfare, the Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases, and Japanese Respiratory Society

Affiliations

¹ Toho University, Faculty of Medicine, Omori Medical Center, Department of Respiratory Medicine, 6-11-1 Omori Nishi, Ota-ku, Tokyo 143-8541, Japan. Electronic address: [email protected].
² Jichi Medical University, Department of Medicine, Division of Pulmonary Medicine, Japan.
³ Nippon Medical School Graduate School of Medicine, Department of Pulmonary Medicine and Oncology, Japan.
⁴ Toho University, Faculty of Medicine, Omori Medical Center, Department of Respiratory Medicine, 6-11-1 Omori Nishi, Ota-ku, Tokyo 143-8541, Japan.
⁵ Dokkyo Medical University, School of Medicine, Department of Pulmonary Medicine and Clinical Immunology, Japan.
⁶ National Center for Global Health and Medicine, Department of Respiratory Medicine, Japan.
⁷ Tokyo Medical and Dental University, Japan.
⁸ National Hospital Organization Kinki, Chuo Chest Medical Center, Clinical Research Center, Japan.
⁹ Tohoku Medical and Pharmaceutical University School of Medicine, Department of Respiratory Medicine, Japan.
¹⁰ Kanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Japan.
¹¹ Toranomon Hospital, Department of Respiratory Medicine, Respiratory Center, Japan.
¹² Okinawa Chubu Hospital, Department of Respiratory Medicine, Japan.
¹³ University of Occupational and Environmental Health, Japan, Department of Respiratory Medicine, Japan.
¹⁴ Kyoto University, Graduate School of Medicine and Public Health, Department of Health Informatics, Japan.
¹⁵ Juntendo University Urayasu Hospital, Department of Respiratory Medicine, Japan.
¹⁶ Kinki Central Hospital of Mutual Aid, Association of Public Schoolteachers, Department of Radiology, Japan.
¹⁷ Hamamatsu University School of Medicine,Second Division, Department of Internal Medicine, Japan.
¹⁸ Juntendo University, Graduate School of Medicine, Department of Respiratory Medicine, Japan.
¹⁹ Sapporo Medical University, School of Medicine, Department of Respiratory Medicine and Allergology, Japan.
²⁰ Tenri Hospital, Department of Respiratory Medicine, Japan.
²¹ Kyoto University, Graduate School of Medicine, Department of Thoracic Surgery, Japan.
²² Tosei General Hospital, Department of Respiratory and Allergic Diseases, Japan.
²³ Tokushima University Graduate School of Medical Sciences, Department of Respiratory Medicine and Rheumatology, Japan.
²⁴ Nagoya University Graduate School of Medicine, Department of Respiratory Medicine, Japan.
²⁵ Hiroshima University, Graduate School of Biomedical and Health Sciences, Department of Molecular and Internal Medicine, Japan.
²⁶ Nagasaki University, Graduate School of Biomedical Sciences, Department of Pathology, Japan.
²⁷ Nagasaki University Graduate School of Biomedical Sciences, Department of Respiratory Medicine, Japan.
²⁸ Kochi University, Kochi Medical School, Department of Hematology and Respiratory Medicine, Japan.
²⁹ Chiba University, Graduate School of Medicine, Department of General Thoracic Surgery, Japan.
³⁰ General Medical Research Center, Fukuoka University School of Medicine, Japan.

PMID: 29980444
DOI: 10.1016/j.resinv.2018.03.003

Abstract

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.

Publication types

Review

MeSH terms

Acetylcysteine / administration & dosage
Administration, Inhalation
Adrenal Cortex Hormones / administration & dosage
Antineoplastic Combined Chemotherapy Protocols / therapeutic use
Comorbidity
Disease Progression
Drug Therapy, Combination
Evidence-Based Medicine
Glycine / administration & dosage
Glycine / analogs & derivatives
Humans
Idiopathic Pulmonary Fibrosis / drug therapy*
Idiopathic Pulmonary Fibrosis / epidemiology
Idiopathic Pulmonary Fibrosis / mortality
Immunosuppressive Agents / administration & dosage
Indoles / administration & dosage
Japan
Lung Neoplasms / drug therapy
Lung Neoplasms / epidemiology
Meta-Analysis as Topic
Practice Guidelines as Topic*
Prognosis
Pyridones / administration & dosage
Quality of Health Care
Sulfonamides / administration & dosage
Survival Rate

Substances

Adrenal Cortex Hormones
Immunosuppressive Agents
Indoles
Pyridones
Sulfonamides
pirfenidone
sivelestat
nintedanib
Glycine
Acetylcysteine