Intractable diffuse pulmonary diseases: Manual for diagnosis and treatment.

This manual has been compiled by a joint production committee with the Diffuse Lung Disease Assembly of the Japanese Respiratory Society (JRS) to provide a practical manual for the epidemiology, diagnosis, and treatment of intractable diffuse pulmonary diseases. The contents are based upon the results of research into these diseases by the Diffuse Pulmonary Diseases Study Group (principal researcher: Sakae Homma) supported by the FY2014-FY2016 Health and Labor Sciences Research Grant on Intractable Diseases. This manual focuses on: 1) pulmonary alveolar microlithiasis, 2) bronchiolitis obliterans, and 3) Hermansky-Pudlak Syndrome with interstitial pneumonia. As these are rare/intractable diffuse lung diseases (2 and 3 were first recognized as specified intractable diseases in 2015), there have not been sufficient epidemiological studies made, and there has been little progress in formulating diagnostic criteria and severity scales; however, the results of Japan's first surveys and research into such details are presented herein. In addition, the manual provides treatment guidance and actual cases for each disease, aiming to assist in the establishment of future modalities. The manual was produced with the goal of enabling clinicians specialized in respiratory apparatus to handle these diseases in clinical settings and of further advancing future research and treatment.

Informations de copyright

Copyright © 2020 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Conflict of Interest The Japanese Respiratory Society has set up a conflicts of interest (COI) committee to manage COI appropriately, based on the shared guidelines and detailed regulations regarding COI determined in coordination with the Japanese Society of Internal Medicine (the guidelines and forms regarding COI are published on the society's website). COI information for members of the Intractable Diffuse Pulmonary Diseases: Manual for Diagnosis and Treatment Production Committee is shown below. Company/organization name: Astellas Pharma Inc.; Eisai Co., Ltd.; LSI Medience Corporation; Ono Pharmaceutical Co., Ltd.; Smoking Research Foundation; GlaxoSmithKline K. K.; Sekisui Medical Co., Ltd.; Taisho Toyama Pharmaceutical Co., Ltd.; Sumitomo Dainippon Pharma Co., Ltd.; Taiho Pharmaceutical Co., Ltd.; Chugai Pharmaceutical Co., Ltd.; Teijin Pharma Ltd.; Toshiba Medical Systems Corp.; Eli Lilly Japan K. K.; Boehringer Ingelheim Japan, Inc.; Bayer Yakuhin, Ltd.; Pfizer Seiyaku K.K. Company/organization name: AstraZeneca K.K.; Ono Pharmaceutical Co., Ltd.; Daiichi Sankyo Co., Ltd.; Taiho Pharmaceutical Co., Ltd.; Chugai Pharmaceutical Co., Ltd.; Boehringer Ingelheim Japan, Inc.; Pfizer Japan Inc. None are applicable to the society's criteria for disclosure.