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

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...

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Autores principales: Nishioka, Yasuhiko, Toyoda, Yuko, Egashira, Ryoko, Johkoh, Takeshi, Terasaki, Yasuhiro, Hebisawa, Akira, Abe, Kinya, Baba, Tomohisa, Fujikura, Yuji, Fujita, Etsuo, Hamada, Naoki, Handa, Tomohiro, Hasegawa, Yoshinori, Hidaka, Koko, Hisada, Takeshi, Hisata, Shu, Honjo, Chisato, Ichikado, Kazuya, Inoue, Yoshikazu, Izumi, Shinyu, Kato, Motoyasu, Kishimoto, Takumi, Okamoto, Masaki, Miki, Keisuke, Mineshita, Masamichi, Nakamura, Yutaro, Sakamoto, Susumu, Sano, Masaaki, Tsukada, Yoshikazu, Yamasue, Mari, Bando, Yoshimi, Homma, Sakae, Hagiwara, Koichi, Suda, Takafumi, Inase, Naohiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9516172/
https://www.ncbi.nlm.nih.gov/pubmed/36162917
http://dx.doi.org/10.1136/bmjresp-2022-001337
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author Nishioka, Yasuhiko
Toyoda, Yuko
Egashira, Ryoko
Johkoh, Takeshi
Terasaki, Yasuhiro
Hebisawa, Akira
Abe, Kinya
Baba, Tomohisa
Fujikura, Yuji
Fujita, Etsuo
Hamada, Naoki
Handa, Tomohiro
Hasegawa, Yoshinori
Hidaka, Koko
Hisada, Takeshi
Hisata, Shu
Honjo, Chisato
Ichikado, Kazuya
Inoue, Yoshikazu
Izumi, Shinyu
Kato, Motoyasu
Kishimoto, Takumi
Okamoto, Masaki
Miki, Keisuke
Mineshita, Masamichi
Nakamura, Yutaro
Sakamoto, Susumu
Sano, Masaaki
Tsukada, Yoshikazu
Yamasue, Mari
Bando, Yoshimi
Homma, Sakae
Hagiwara, Koichi
Suda, Takafumi
Inase, Naohiko
author_facet Nishioka, Yasuhiko
Toyoda, Yuko
Egashira, Ryoko
Johkoh, Takeshi
Terasaki, Yasuhiro
Hebisawa, Akira
Abe, Kinya
Baba, Tomohisa
Fujikura, Yuji
Fujita, Etsuo
Hamada, Naoki
Handa, Tomohiro
Hasegawa, Yoshinori
Hidaka, Koko
Hisada, Takeshi
Hisata, Shu
Honjo, Chisato
Ichikado, Kazuya
Inoue, Yoshikazu
Izumi, Shinyu
Kato, Motoyasu
Kishimoto, Takumi
Okamoto, Masaki
Miki, Keisuke
Mineshita, Masamichi
Nakamura, Yutaro
Sakamoto, Susumu
Sano, Masaaki
Tsukada, Yoshikazu
Yamasue, Mari
Bando, Yoshimi
Homma, Sakae
Hagiwara, Koichi
Suda, Takafumi
Inase, Naohiko
author_sort Nishioka, Yasuhiko
collection PubMed
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 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.
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spelling pubmed-95161722022-09-29 Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype Nishioka, Yasuhiko Toyoda, Yuko Egashira, Ryoko Johkoh, Takeshi Terasaki, Yasuhiro Hebisawa, Akira Abe, Kinya Baba, Tomohisa Fujikura, Yuji Fujita, Etsuo Hamada, Naoki Handa, Tomohiro Hasegawa, Yoshinori Hidaka, Koko Hisada, Takeshi Hisata, Shu Honjo, Chisato Ichikado, Kazuya Inoue, Yoshikazu Izumi, Shinyu Kato, Motoyasu Kishimoto, Takumi Okamoto, Masaki Miki, Keisuke Mineshita, Masamichi Nakamura, Yutaro Sakamoto, Susumu Sano, Masaaki Tsukada, Yoshikazu Yamasue, Mari Bando, Yoshimi Homma, Sakae Hagiwara, Koichi Suda, Takafumi Inase, Naohiko BMJ Open Respir Res Orphan Lung Disease 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. BMJ Publishing Group 2022-09-26 /pmc/articles/PMC9516172/ /pubmed/36162917 http://dx.doi.org/10.1136/bmjresp-2022-001337 Text en © 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. https://creativecommons.org/licenses/by-nc/4.0/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/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Orphan Lung Disease
Nishioka, Yasuhiko
Toyoda, Yuko
Egashira, Ryoko
Johkoh, Takeshi
Terasaki, Yasuhiro
Hebisawa, Akira
Abe, Kinya
Baba, Tomohisa
Fujikura, Yuji
Fujita, Etsuo
Hamada, Naoki
Handa, Tomohiro
Hasegawa, Yoshinori
Hidaka, Koko
Hisada, Takeshi
Hisata, Shu
Honjo, Chisato
Ichikado, Kazuya
Inoue, Yoshikazu
Izumi, Shinyu
Kato, Motoyasu
Kishimoto, Takumi
Okamoto, Masaki
Miki, Keisuke
Mineshita, Masamichi
Nakamura, Yutaro
Sakamoto, Susumu
Sano, Masaaki
Tsukada, Yoshikazu
Yamasue, Mari
Bando, Yoshimi
Homma, Sakae
Hagiwara, Koichi
Suda, Takafumi
Inase, Naohiko
Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title_full Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title_fullStr Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title_full_unstemmed Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title_short Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
title_sort nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
topic Orphan Lung Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9516172/
https://www.ncbi.nlm.nih.gov/pubmed/36162917
http://dx.doi.org/10.1136/bmjresp-2022-001337
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