RT Journal Article
SR Electronic
T1 Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
JF BMJ Open Respiratory Research
JO BMJ Open Resp Res
FD British Thoracic Society
SP e001337
DO 10.1136/bmjresp-2022-001337
VO 9
IS 1
A1 Yasuhiko Nishioka
A1 Yuko Toyoda
A1 Ryoko Egashira
A1 Takeshi Johkoh
A1 Yasuhiro Terasaki
A1 Akira Hebisawa
A1 Kinya Abe
A1 Tomohisa Baba
A1 Yuji Fujikura
A1 Etsuo Fujita
A1 Naoki Hamada
A1 Tomohiro Handa
A1 Yoshinori Hasegawa
A1 Koko Hidaka
A1 Takeshi Hisada
A1 Shu Hisata
A1 Chisato Honjo
A1 Kazuya Ichikado
A1 Yoshikazu Inoue
A1 Shinyu Izumi
A1 Motoyasu Kato
A1 Takumi Kishimoto
A1 Masaki Okamoto
A1 Keisuke Miki
A1 Masamichi Mineshita
A1 Yutaro Nakamura
A1 Susumu Sakamoto
A1 Masaaki Sano
A1 Yoshikazu Tsukada
A1 Mari Yamasue
A1 Yoshimi Bando
A1 Sakae Homma
A1 Koichi Hagiwara
A1 Takafumi Suda
A1 Naohiko Inase
YR 2022
UL http://bmjopenrespres.bmj.com/content/9/1/e001337.abstract
AB 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.All data relevant to the study are included in the article or uploaded as online supplemental information.