Reisi M, Azizi G, Kiaee F, Masiha F, Shirzadi R, Momen T, Rafiemanesh H, Tavakolinia N, Modaresi M, Aghamohammadi A
Pediatric Pulmonology Department, Child Growth and Development Research Center, Research Institute of Primordial Prevention of non-communable Disease, Isfahan University of medical sciences, Isfahan, Iran.
Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Eur Ann Allergy Clin Immunol. 2017 May;49(3):122-128.
Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of immune system are defected. Moreover, affected patients are at high risk for developing recurrent infections, particularly pulmonary infections. The spectrum of pulmonary manifestations in PIDs is broad, and includes acute and chronic infection, structural abnormalities (eg, bronchiectasis), malignancy and dysregulated inflammation resulting in tissue damage. In this study, our aims are to evaluate pulmonary complications in PID patients. We studied 204 cases with confirmed PID. To evaluate pulmonary complications in these patients, we used pulmonary function test (PFT), high resolution computed tomography (HRCT) scan and bronchoalveolar lavage (BAL). Our results showed that pneumonia was the most frequent clinical manifestations in all PID patients. There were significantly greater numbers of episodes of pneumonia in HIgM, XLA and CVID patients with delayed diagnoses < 6 years. Moreover, of 57.4% CVID patients, 55% XLA patients and 33.3% HIgM patients had abnormal PFT results, and bronchiectasis was showed in 9 (42.9%) of XLA, 6 (11.8%) of HIES, 3 (21.4%) of HIgM and 38 (62.3%) of CVID patients. Pulmonary complications should be considered in cases with PIDs especially in CVID cases.
原发性免疫缺陷病（PIDs）是遗传性疾病，其中免疫系统的一个或几个组成部分存在缺陷。此外，受影响的患者发生反复感染，尤其是肺部感染的风险很高。PIDs的肺部表现范围广泛，包括急性和慢性感染、结构异常（如支气管扩张）、恶性肿瘤以及导致组织损伤的炎症调节异常。在本研究中，我们的目的是评估PID患者的肺部并发症。我们研究了204例确诊为PID的病例。为了评估这些患者的肺部并发症，我们使用了肺功能测试（PFT）、高分辨率计算机断层扫描（HRCT）和支气管肺泡灌洗（BAL）。我们的结果表明，肺炎是所有PID患者中最常见的临床表现。在诊断延迟<6年的高IgM血症、X连锁无丙种球蛋白血症（XLA）和常见变异型免疫缺陷病（CVID）患者中，肺炎发作的次数明显更多。此外，57.4%的CVID患者、55%的XLA患者和33.3%的高IgM血症患者的PFT结果异常，XLA患者中有9例（42.9%）、高IgE综合征（HIES）患者中有6例（11.8%）、高IgM血症患者中有3例（21.4%）以及CVID患者中有38例（62.3%）出现支气管扩张。对于PID患者，尤其是CVID患者，应考虑肺部并发症。
