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Intractable diffuse pulmonary diseases: Manual for diagnosis and treatment
Authors:Sakae Homma  Masahito Ebina  Kazuyoshi Kuwano  Hisatsugu Goto  Fumikazu Sakai  Susumu Sakamoto  Takeshi Johkoh  Keishi Sugino  Teruo Tachibana  Yasahiro Terasaki  Yasuhiko Nishioka  Koichi Hagiwara  Naozumi Hashimoto  Yoshinori Hasegawa  Akira Hebisawa
Affiliation:1. Department of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Tokyo, Japan;2. Department of Respiratory Medicine in the 1st Internal Medicine, Tohoku Medical and Pharmaceutical University School of Medicine, Sendai, Japan;3. Division of Respiratory Diseases, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan;4. Department of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan;5. Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, Saitama, Japan;6. Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan;7. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Hyogo, Japan;8. Department of Internal Medicine, Aizenbashi Hospital, Osaka, Japan;9. Department of Pathology (Analytic Human Pathology), Nippon Medical School, Tokyo, Japan;10. Division of Pulmonary Medicine, Jichi Medical University, Saitama, Japan;11. Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan;12. National Hospital Organization Tokyo Medical Center, Tokyo, Japan;1. Department of Respiratory Medicine and Infectious Disease, Yamaguchi University, Ube, Japan;2. Department of Pulmonary Medicine, Tokai University Tokyo Hospital, Tokyo, Japan;3. First Department of Internal Medicine, Shinshu University, Matsumoto, Japan;4. Department of Pulmonary Medicine, Fukushima Medical University, Fukushima, Japan;5. Division of Respiratory Diseases, Saint Marianna University Yokohama City Seibu Hospital, Yokohama, Japan;6. Division of Pulmonary Medicine, Department of Medicine, Keio University, Tokyo, Japan;7. Department of Respiratory Medicine, Kyoto University, Kyoto, Japan;8. Department of Respiratory Medicine, Tohoku University, Sendai, Japan;1. Department of Clinical Mycobacteriology, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan;2. Respiratory Care and Rehabilitation Center, Fukujuji Hospital, Japan Anti-Tuberculosis Association, 3-1-24 Matsuyama, Kiyose-shi, Tokyo, 204-8522, Japan;3. Department of Rehabilitation, Kanamecho Hospital, 1-11-13 Kanamecho, Toshima-ku, Tokyo, 171-0043, Japan;1. Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan, 5-1-1 Nabeshima, Saga, Saga prefecture, 849-8501, Japan;2. Department of Otolaryngology, Head & Neck Surgery, Faculty of Medicine, Saga University, Saga, Japan, 5-1-1 Nabeshima, Saga, Saga prefecture, 849-8501, Japan;1. Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Tochigi, Japan;2. Division of Pulmonary Medicine, Department of Thoracic Surgery, Jichi Medical University, Tochigi, Japan;1. Division of Laboratory and Transfusion Medicine, Hokkaido University Hospital, Sapporo, Hokkaido, Japan;2. Department of Respiratory Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaido, Japan
Abstract: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.
Keywords:Pulmonary alveolar microlithiasis  Bronchiolitis obliterans  Hermansky-Pudlak syndrome  Interstitial pneumonia  Intractable diffuse pulmonary diseases
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