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ID 118569
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Toyoda, Yuko Tokushima University|Japanese Red Cross Kochi Hospital KAKEN Search Researchers
Egashira, Ryoko Saga University
Johkoh, Takeshi Kansai Rosai Hospital
Terasaki, Yasuhiro Nippon Medical School
Hebisawa, Akira Asahi General Hospital
Abe, Kinya Toyonaka Municipal Hospital
Baba, Tomohisa Kanagawa Cardiovascular and Respiratory Center
Fujikura, Yuji National Defense Medical College
Fujita, Etsuo Hashimoto Municipal Hospital
Hamada, Naoki Kyushu University
Handa, Tomohiro Kyoto University
Hasegawa, Yoshinori Osaka Saiseikai Nakatsu Hospital
Hidaka, Koko National Hospital Organization Kokura Medical Center
Hisada, Takeshi Gunma University
Hisata, Shu Jichi Medical University
Honjo, Chisato University of Fukui
Ichikado, Kazuya Saiseikai Kumamoto Hospital
Inoue, Yoshikazu National Hospital Organization Kinki-Chuo Chest Medical Center
Izumi, Shinyu National Center for Global Health and Medicine
Kato, Motoyasu Juntendo University
Kishimoto, Takumi Okayama Rosai Hospital
Okamoto, Masaki Kurume University
Miki, Keisuke National Hospital Organization Osaka Toneyama Medical Center
Mineshita, Masamichi St.Marianna University School of Medicine
Nakamura, Yutaro Hamamatsu University School of Medicine
Sakamoto, Susumu Toho University
Sano, Masaaki Akita University
Tsukada, Yoshikazu Soka Municipal Hospital
Yamasue, Mari Oita University
Homma, Sakae Toho University
Hagiwara, Koichi Jichi Medical University
Suda, Takafumi Hamamatsu University School of Medicine
Inase, Naohiko Hiratsuka Kyosai Hospital
Content Type
Journal Article
Description
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 1s). 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 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed.
)) at diagnosis. Nearly 80% of the subjects wereasymptomatic, 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 1s). 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.
Journal Title
BMJ Open Respiratory Research
ISSN
20524439
Publisher
BMJ Publishing Group
Volume
9
Issue
1
Start Page
e001337
Published Date
2022-09-26
Rights
This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
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language
eng
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Medical Sciences
University Hospital