Diffuse pulmonary ossification and its association with cicatricial organising pneumonia in idiopathic and secondary forms

Diffuse pulmonary ossification (DiPO) is characterised by widespread ectopic bone formation in the lungs. Idiopathic DiPO (I-DiPO) poses significant diagnostic challenges and its ossification mechanism remains unclear. Cicatricial organising pneumonia (CiOP) lesions form fibrous nodules without damaging lung structure. We investigated the histopathological features of I-DiPO, focusing on the surrounding fibrosis, and compared them with those of secondary DiPO (S-DiPO). An analysis was conducted using data from a nationwide DiPO survey in Japan. The dataset included clinical, radiological, and histopathological data of patients with suspected I-DiPO. The specific patterns of ossification and fibrotic findings such as CiOP, organising pneumonia (OP), and subpleural fibrosis were identified. Eighteen and seven patients were classified as having I-DiPO and S-DiPO, respectively. I-DiPO affects younger patients, progresses slowly, commonly occurs in the lower lungs, and has a lower mortality rate. S-DiPO affects older patients, presents with widespread lung lesions, and has a higher mortality rate. CiOP lesions were found in direct continuity with or near ossified lesions in 61.1% and 71.4% of patients with I-DiPO and S-DiPO, respectively. OP, CiOP, and ossified lesions often observed in the same locations in S-DiPO. DiPO has a unique pathogenesis, with an ossification transition occurring via the CiOP lesions. These findings provide valuable insights for future diagnostic approaches and management strategies for this condition.

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Publication Details

Journal
Scientific Reports
Published
2025-04-11
DOI
https://doi.org/10.1038/s41598-025-95307-0
Primary Topic
Medical Imaging and Pathology Studies
Type
article
Field-Weighted Citation Impact
0.00

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article

Diffuse pulmonary ossification and its association with cicatricial organising pneumonia in idiopathic and secondary forms

Yoshikazu Inoue, Mika Terasaki, Masamichi Mineshita, Mari Yamasue et al.
Scientific Reports
Medical Imaging and Pathology Studies
article

Diffuse pulmonary ossification and its association with cicatricial organising pneumonia in idiopathic and secondary forms

Yoshikazu Inoue, Mika Terasaki, Masamichi Mineshita, Mari Yamasue, Takeshi Johkoh, Shu Hisata, Kazuya Ichikado, Yuko Toyoda, Yasuhiko Nishioka, Koko Hidaka, Takumi Kishimoto, Motoyasu Kato, Shinyu Izumi, Ryoko Egashira, Chisato Honjo, Tomohiro Handa, Yasuhiro Terasaki, Akira Hebisawa, Yuji Fujikura, Takeshi Hisada, Masaki Okamoto, Tomohisa Baba, Yoshinori Hasegawa, Takafumi Suda, Yusuke Kajimoto
article en

Abstract

Diffuse pulmonary ossification (DiPO) is characterised by widespread ectopic bone formation in the lungs. Idiopathic DiPO (I-DiPO) poses significant diagnostic challenges and its ossification mechanism remains unclear. Cicatricial organising pneumonia (CiOP) lesions form fibrous nodules without damaging lung structure. We investigated the histopathological features of I-DiPO, focusing on the surrounding fibrosis, and compared them with those of secondary DiPO (S-DiPO). An analysis was conducted using data from a nationwide DiPO survey in Japan. The dataset included clinical, radiological, and histopathological data of patients with suspected I-DiPO. The specific patterns of ossification and fibrotic findings such as CiOP, organising pneumonia (OP), and subpleural fibrosis were identified. Eighteen and seven patients were classified as having I-DiPO and S-DiPO, respectively. I-DiPO affects younger patients, progresses slowly, commonly occurs in the lower lungs, and has a lower mortality rate. S-DiPO affects older patients, presents with widespread lung lesions, and has a higher mortality rate. CiOP lesions were found in direct continuity with or near ossified lesions in 61.1% and 71.4% of patients with I-DiPO and S-DiPO, respectively. OP, CiOP, and ossified lesions often observed in the same locations in S-DiPO. DiPO has a unique pathogenesis, with an ossification transition occurring via the CiOP lesions. These findings provide valuable insights for future diagnostic approaches and management strategies for this condition.

Scientific ReportsVol. 15(1)
Kurume University (JP), Jichi Medical University (JP), St. Marianna University School of Medicine (JP), Gunma University (JP), Saga University (JP), Oita University (JP), Kyoto University (JP), Hamamatsu University School of Medicine (JP), Juntendo University (JP), National Center for Global Health and Medicine (JP), Saiseikai Kumamoto Hospital (JP), University of Fukui Hospital (JP), Kansai Rosai Hospital (JP), Japanese Red Cross Kochi Hospital (JP), Saiseikai Nakatsu Hospital (JP), Kanagawa Cardiovascular and Respiratory Center (JP), Okayama Rosai Hospital (JP), Kyoto University Hospital (JP), Tokyo National Hospital (JP), NHO Kinki Chuo Chest Medical Center (JP), Kokura Memorial Hospital (JP), ShinKokura Hospital (JP), National Defense Medical College (JP), Nippon Medical School (JP), Tokushima University (JP)
Ministry of Education, Culture, Sports, Science and Technology
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Openalex Percentile: Top 88%
Medical Imaging and Pathology Studies
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