PT  - JOURNAL ARTICLE
AU  - Hans Slabbynck
AU  - Tom De Beukelaar
AU  - Filip Hoet
AU  - Didier De Surgeloose
AU  - Karina Charels
AU  - Jozef Van Goethem
TI  - Diffuse dendriform pulmonary ossification in a usual interstitial pneumonia-like distribution: A distinct entity or a variant of idiopathic pulmonary fibrosis?
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P737
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P737.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P737.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Diffuse dendriform pulmonary ossificiation (DPO) is a rare disease characterized by diffuse small bone fragments in lung tissue. Diagnosis is revealed by a coral-like, branching, dendritic pattern of bone density on high resolution CT (HRCT) with osteoporosis window settings. Dendriform DPO is associated with chronic pulmonary disorders.HRCT criteria for a definite usual interstitial pneumonia (UIP) pattern include a predominantly subpleural and basilar distribution of reticular pattern in the absence of inconsistent features and associated with honeycombing. A possible UIP pattern is identical with the omission of honeycombing.There are only a handful of case reports of DPO with a UIP-like distribution diagnosed during life. In older autopsy series the prevalence of DPO features in IPF is as high as 9 %. In a recent study of 10426 autopsy cases, there were 5 cases of dendriform DPO with a UIP distribution. In a HRCT study, mild DPO was identified in 5 of 75 patients with UIP.We report three elderly men in whom HRCT revealed the diagnosis of extensive dendriform DPO in a UIP-distribution. They were former smokers, one had exposure as a metal worker. In one patient autopsy subsequently confirmed the diagnosis of DPO. Two patients are brothers, suggesting a genetic predisposition.The slowly progressive nature of the disorder contrasting with idiopathic pulmonary fibrosis (IPF), the absence of radiological honeycombing despite longlasting disease, and the highly likelihood of a genetic predisposition suggest that DPO in a UIP-like distribution is at least a variant phenotype of IPF or might be just a different entity.