Slabbynck Hans, de Beukelaar Tom, De Surgeloose Didier, Van Goethem Jozef, Charels Karina, Bedert Lieven, Wuyts Wim
Middelheim General Hospital, ZNA, Dept. Respiratory Medicine, Antwerp, BE.
ZNA, Dept Radiology, Antwerp, BE.
Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(3):251-256. doi: 10.36141/svdld.v34i3.6032. Epub 2020 Mar 9.
Idiopathic dendriform diffuse pulmonary ossification (DPO) is a rare disorder. High resolution CT (HRCT) with appropriate osteoporosis window setting reveals the diagnosis. We report the features of eight patients, of whom two brothers, with HRCT findings compatible with predominant DPO in a bibasal subpleural distribution (usual interstitial pneumonia (UIP)-like distribution) and review the literature for DPO in this UIP-like distribution. DPO in a UIP-like distribution seems to be a disorder of the very old (age 75-87 (mean 83.6) male (8 out of 8), with familial occurrence, with associated cardiovascular disease and frequent use of anticoagulants as common findings, and with a slowly progressive nature and the absence of radiological honeycombing despite long lasting disease contrasting with idiopathic pulmonary fibrosis (IPF). Idiopathic pulmonary fibrosis (IPF) should be differentiated from predominant DPO in a UIP-like distibution. .
特发性树枝状弥漫性肺骨化（DPO）是一种罕见的疾病。采用适当的骨质疏松窗设置的高分辨率CT（HRCT）可明确诊断。我们报告了8例患者的特征，其中包括两兄弟，其HRCT表现符合双肺基底胸膜下为主的DPO（类似寻常型间质性肺炎（UIP）的分布），并回顾了有关这种类似UIP分布的DPO的文献。类似UIP分布的DPO似乎是一种多见于老年人（年龄75 - 87岁（平均83.6岁），男性（8例均为男性））的疾病，具有家族性发病特点，常见合并心血管疾病和频繁使用抗凝剂，病程呈缓慢进展，尽管疾病持续时间长但无放射学蜂窝肺表现，这与特发性肺纤维化（IPF）不同。特发性肺纤维化（IPF）应与类似UIP分布的为主型DPO相鉴别。
