Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype.

Autor:	Nishioka Y; Department of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan yasuhiko@tokushima-u.ac.jp., Toyoda Y; Department of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan.; Department of Internal Medicine, Japanese Red Cross Kochi Hospital, Kochi, Japan., Egashira R; Department of Radiology, Faculty of Medicine, Saga University, Saga, Japan., Johkoh T; Department of Radiology, Kansai Rosai Hospital, Amagasaki, Hyogo, Japan., Terasaki Y; Department of Analytic Human Pathology, Nippon Medical School, Bunkyo-ku, Tokyo, Japan., Hebisawa A; Department of Clinical Pathology, Asahi General Hospital, Chiba, Japan., Abe K; Department of Internal Medicine, Toyonaka Municipal Hospital, Toyonaka, Osaka, Japan., Baba T; Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan., Fujikura Y; Division of Infectious Diseases and Respiratory Medicine, Department of Internal Medicine, National Defense Medical College, Tokorozawa, Saitama, Japan., Fujita E; Department of Respiratory Medicine, Hashimoto Municipal Hospital, Hashimoto, Wakayama, Japan., Hamada N; Research Institute for Diseases of the Chest, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Handa T; Department of Advanced Medicine for Respiratory Failure, Graduate School of Medicine, Kyoto University, Kyoto, Japan., Hasegawa Y; Department of Respiratory Medicine, Osaka Saiseikai Nakatsu Hospital, Osaka, Japan., Hidaka K; Department of Respiratory Medicine, National Hospital Organization Kokura Medical Center, Kitakyushu, Fukuoka, Japan., Hisada T; Department of Respiratory Medicine, Gunma University Graduate School of Medicine, Meabashi, Gunma, Japan., Hisata S; Division of Pulmonary Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan., Honjo C; Department of Respiratory Medicine, University of Fukui Hospital, Fukui, Japan., Ichikado K; Department of Respiratory Medicine, Saiseikai Kumamoto Hospital, Kumamoto, Japan., Inoue Y; Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan., Izumi S; Department of Respiratory Medicine, National Center for Global Health and Medicine, Tokyo, Japan., Kato M; Department of Respiratory Medicine, Graduate School of Medicine, Juntendo University, Tokyo, Japan., Kishimoto T; Department of Medicine, Okayama Rosai Hospital, Okayama, Japan., Okamoto M; Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Kurume, Fukuoka, Japan., Miki K; Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center, Toyonaka, Osaka, Japan., Mineshita M; Division of Respiratory Medicine, Department of Internal Medicine, St.Marianna University School of Medicine, Kawasaki, Kanagawa, Japan., Nakamura Y; Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan., Sakamoto S; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan., Sano M; Department of Respiratory Medicine, Akita University Graduate School of Medicine, Akita, Japan., Tsukada Y; Department of Respiratory Medicine, Soka Municipal Hospital, Soka, Saitama, Japan., Yamasue M; Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine, Yufu, Oita, Japan., Bando Y; Division of Pathology, Tokushima University Hospital, Tokushima, Japan., Homma S; Department of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Tokyo, Japan., Hagiwara K; Division of Pulmonary Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan., Suda T; Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan., Inase N; Hiratsuka Kyosai Hospital, Federation of National Public Service Personnel Mutual Aid Associations, Hiratsuka, Kanagawa, Japan.
Jazyk:	angličtina
Zdroj:	BMJ open respiratory research [BMJ Open Respir Res] 2022 Sep; Vol. 9 (1).
DOI:	10.1136/bmjresp-2022-001337
Abstrakt:	Background: Diffuse pulmonary ossification is a specific lung condition that is accompanied by underlying diseases. However, idiopathic dendriform pulmonary ossification (IDPO) is extremely rare, and the clinical features remain unclear. In this study, we aimed to report the clinical characteristics of IDPO. Methods: We conducted a nationwide survey of patients with IDPO from 2017 to 2019 in Japan and evaluated the clinical, radiological, and histopathological findings of patients diagnosed with IDPO. Results: Twenty-two cases of IDPO were identified. Most subjects (82%) were male, aged 22-56 years (mean (SD), 37.9 (9.1)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1 s). Two cases with rapid decline of 10% /year in %FVC predicted were observed.)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1 s). Two cases with rapid decline of 10% /year in %FVC predicted were observed. Conclusions: IDPO develops at a young age with gradually progressive phenotype. Further research and long-term (>20 years) follow-up are required to clarify the pathogenesis and clinical findings in IDPO. Competing Interests: Competing interests: YI is a consultant and steering committee member to Boehringer Ingelheim, Roche, Taiho, and Savara, and has a lecture fee from Boehringer Ingelheim, Shionogi, Kyorin and Thermo Fisher. (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze:	MEDLINE
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