Yale University School of Medicine, New Haven, CT, USA.
Section of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.
BMC Pulm Med. 2019 Feb 8;19(1):34. doi: 10.1186/s12890-019-0797-7.
Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder defined by high serum immunoglobulin E titers that is associated with recurrent respiratory infections, formation of pneumoatoceles, recurrent skin abscesses, and characteristic dental and skeletal abnormalities.
We report a case of a 56-year-old male with a history of HIES, cavitary mycetomas, and allergic bronchopulmonary aspergillosis who presented with recurrent massive hemoptysis. Bronchial artery angiography and bronchoscopy failed to identify active hemorrhage, and two embolizations of the bronchial artery did not resolve the bleeding. Subsequently, selective pulmonary artery angiography was conducted that demonstrated a subsegmental pulmonary artery branch pseudoaneurysm with extravasation into an adjacent lung cavity. This was treated successfully with transcatheter embolization.
To our knowledge, this is the first case reported of pulmonary artery pseudoaneurysm in HIES in the medical literature. Pulmonary artery pseudoaneurysm should be considered in the differential diagnosis in patients with HIES and massive hemoptysis.
高免疫球蛋白 E 综合征（HIES）是一种罕见的原发性免疫缺陷病，其特征为血清免疫球蛋白 E 滴度升高，常伴有复发性呼吸道感染、肺大疱形成、复发性皮肤脓肿以及特征性的牙齿和骨骼异常。
我们报告了 1 例 56 岁男性病例，该患者有 HIES、腔隙性真菌肿和变应性支气管肺曲霉病病史，表现为反复大量咯血。支气管动脉血管造影和支气管镜检查未能确定活动性出血，两次支气管动脉栓塞也未能解决出血问题。随后进行了选择性肺动脉造影，显示亚段肺动脉分支假性动脉瘤，有血液外渗到相邻的肺腔。经导管栓塞治疗后成功治愈。
据我们所知，这是医学文献中首例报道的 HIES 肺动脉假性动脉瘤病例。对于 HIES 合并大量咯血的患者，应考虑肺动脉假性动脉瘤作为鉴别诊断。
