Damadoğlu Ebru
Division of Allergy and Clinical Immunology, Department of Pulmonology, Hacettepe University School of Medicine, Ankara, Turkey.
Turk Thorac J. 2021 May;22(3):257-264. doi: 10.5152/TurkThoracJ.2021.20097.
Primary antibody deficiency diseases result from a genetic defect that causes misfunction of 1 or more of the immune system elements. Due to the increased awareness among physicians and the success of new treatment modalities, the number of pediatric patients reaching adult age and the number of patients diagnosed in adult age is increasing. Adult patients comprise more than half of the total cases. Primary antibody deficiencies are the most common immunodeficiency type in adults, and these may cause recurrent upper and lower respiratory tract infections and result in the development of bronchiectasis. Among non-infectious pulmonary complications, any type of interstitial lung disease may be seen; however, a special type seen in patients with common variable immunodeficiency, namely granulomatous lymphocytic interstitial lung disease, is the one most commonly investigated. Underlying or accompanying immunodeficiency may be present in patients with asthma and chronic obstructive pulmonary disease, especially if the disease requires frequent hospitalizations and/or is severe. Early diagnosis and appropriate management of primary antibody deficiency diseases in patients with respiratory symptoms are crucial to decrease complications and increase survival.
原发性抗体缺陷病是由一种遗传缺陷导致的，这种缺陷会引起一种或多种免疫系统元件功能异常。由于医生的认识提高以及新治疗方式的成功，达到成年年龄的儿科患者数量以及在成年期被诊断出的患者数量正在增加。成年患者占总病例数的一半以上。原发性抗体缺陷是成年人中最常见的免疫缺陷类型，这些缺陷可能导致反复的上呼吸道和下呼吸道感染，并导致支气管扩张的发生。在非感染性肺部并发症中，可能会出现任何类型的间质性肺疾病；然而，在常见可变免疫缺陷患者中出现的一种特殊类型，即肉芽肿性淋巴细胞间质性肺疾病，是最常被研究的类型。哮喘和慢性阻塞性肺疾病患者可能存在潜在的或伴随的免疫缺陷，特别是如果疾病需要频繁住院和/或病情严重时。对有呼吸道症状的患者进行原发性抗体缺陷病的早期诊断和适当管理对于减少并发症和提高生存率至关重要。
