학술논문
Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype
Document Type
article
Author
Yoshinori Hasegawa; Yasuhiko Nishioka; Shinyu Izumi; Sakae Homma; Yoshikazu Inoue; Takumi Kishimoto; Yutaro Nakamura; Takafumi Suda; Kazuya Ichikado; Takeshi Johkoh; Naoki Hamada; Koichi Hagiwara; Akira Hebisawa; Naohiko Inase; Ryoko Egashira; Kinya Abe; Masaki Okamoto; Motoyasu Kato; Yasuhiro Terasaki; Yuko Toyoda; Tomohisa Baba; Yuji Fujikura; Etsuo Fujita; Tomohiro Handa; Koko Hidaka; Takeshi Hisada; Shu Hisata; Chisato Honjo; Keisuke Miki; Masamichi Mineshita; Susumu Sakamoto; Masaaki Sano; Yoshikazu Tsukada; Mari Yamasue; Yoshimi Bando
Source
BMJ Open Respiratory Research, Vol 9, Iss 1 (2022)
Subject
Language
English
ISSN
2052-4439
Abstract
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 20 years) follow-up are required to clarify the pathogenesis and clinical findings in IDPO.