Jończyk-Potoczna Katarzyna, Szczawińska-Popłonyk Aleksandra, Warzywoda Małgorzata, Bręborowicz Anna, Pawlak Bogdan
Pediatric Radiology Department, Chair of Radiology, Poznań University of Medical Sciences, Poznań, Poland.
Pol J Radiol. 2012 Apr;77(2):69-72. doi: 10.12659/pjr.882974.
Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum.
The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations - fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up.
Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome.
高免疫球蛋白E综合征（高IgE综合征、乔布综合征、HIES）是一种具有多器官临床表现和多样遗传背景的复杂免疫缺陷病。在大约75%的HIES患者中观察到的临床三联征症状包括：葡萄球菌病因的复发性脓肿、反复呼吸道感染和血清免疫球蛋白E升高。
本文讨论了3例出现高IgE综合征典型肺部并发症的女性患者。第一例中，观察到炎症后囊肿内曲霉菌球的形成；另一例中，出现肺炎伴胸腔积液，炎症浸润导致纤维化改变，进而进行了肺叶切除术；在最后一例中，肺部疾病的病程因葡萄球菌脓肿的形成而复杂化。其中一名女孩在随访时出现支气管扩张。
肺部感染并发症是高IgE综合征最常见的死亡原因。延迟诊断会显著恶化呼吸功能，并降低儿童正常发育的机会。引入包括预防在内的综合治疗可减少复发。因此，放射科医生在该综合征患者的多学科护理中具有重要作用。
