Zo Sungmin, Moon Ji-Yong, Min Kyung Hoon, Lee Hyun
Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea.
Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Republic of Korea.
Tuberc Respir Dis (Seoul). 2024 Oct;87(4):440-450. doi: 10.4046/trd.2024.0015. Epub 2024 Aug 14.
Bronchiectasis is a chronic respiratory disease characterized by abnormal dilation of the bronchi that causes cough, sputum, and recurrent infections. As it may be associated with various respiratory or systemic diseases, a critical aspect of managing bronchiectasis is to identify the underlying cause. Immunodeficiency is a rare but important cause of bronchiectasis, and its treatability is a significant trait for bronchiectasis management. While primary immunodeficiencies in bronchiectasis are well recognized, secondary immunodeficiencies remain under-reported and under-researched. Secondary immunodeficiencies may result from various diseases and conditions, such as hematologic malignancies, human immunodeficiency virus infection, renal transplantation, or the use of immunosuppressive drugs, and may contribute to the occurrence of bronchiectasis. Recurrent pulmonary and/or extrapulmonary infections in bronchiectasis may indicate the presence of secondary immunodeficiency in patients with these underlying conditions. For treatment, examining the underlying condition, managing bronchiectasis adequately, and prophylactic antibiotics (e.g., macrolide) and/or supplementary immunoglobulin G therapy may provide potential benefits. Considering the projected increase in the prevalence of secondary immunodeficiencies and bronchiectasis, future guidelines and research on the diagnosis and optimized treatment are needed.
支气管扩张是一种慢性呼吸道疾病,其特征为支气管异常扩张,可导致咳嗽、咳痰及反复感染。由于它可能与多种呼吸道或全身性疾病相关,因此管理支气管扩张的一个关键方面是确定潜在病因。免疫缺陷是支气管扩张的一种罕见但重要的病因,其可治疗性是支气管扩张管理的一个重要特征。虽然支气管扩张中的原发性免疫缺陷已得到充分认识,但继发性免疫缺陷的报告和研究仍然不足。继发性免疫缺陷可能由多种疾病和状况引起,如血液系统恶性肿瘤、人类免疫缺陷病毒感染、肾移植或使用免疫抑制药物等,并且可能促成支气管扩张的发生。支气管扩张患者反复发生肺部和/或肺外感染可能提示这些潜在疾病患者存在继发性免疫缺陷。对于治疗,检查潜在疾病、充分管理支气管扩张以及预防性使用抗生素(如大环内酯类)和/或补充免疫球蛋白G治疗可能会带来潜在益处。考虑到继发性免疫缺陷和支气管扩张的患病率预计会上升,未来需要关于诊断和优化治疗的指南及研究。