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

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.