Division of Allergy Immunology, Department of Pediatrics, Louisiana State University Health Sciences Center New Orleans, New Orleans, LA, United States.
Children's Hospital of New Orleans, New Orleans, LA, United States.
Front Immunol. 2020 Mar 31;11:522. doi: 10.3389/fimmu.2020.00522. eCollection 2020.
Bronchiectasis, the presence of bronchial wall thickening with airway dilatation, is a particularly challenging complication of primary antibody deficiencies. While susceptibility to infections may be the primary factor leading to the development of bronchiectasis in these patients, the condition may develop in the absence of known infections. Once bronchiectasis is present, the lungs are subject to a progressive cycle involving both infectious and non-infectious factors. If bronchiectasis is not identified or not managed appropriately, the cycle proceeds unchecked and yields advanced and permanent lung damage. Severe symptoms may limit exercise tolerance, require frequent hospitalizations, profoundly impair quality of life (QOL), and lead to early death. This review article focuses on the appropriate identification and management of bronchiectasis in patients with primary antibody deficiencies. The underlying immune deficiency and the bronchiectasis need to be treated from combined immunology and pulmonary perspectives, reflected in this review by experts from both fields. An aggressive multidisciplinary approach may reduce exacerbations and slow the progression of permanent lung damage.
支气管扩张症是指支气管壁增厚伴气道扩张，是原发性抗体缺陷的一种特别具有挑战性的并发症。尽管感染易感性可能是导致这些患者发生支气管扩张的主要因素，但在没有明确感染的情况下，这种情况也可能发生。一旦发生支气管扩张，肺部就会受到一个涉及感染和非感染因素的进行性循环的影响。如果支气管扩张未被识别或未得到适当管理，该循环将不受控制地进行，导致严重和永久性的肺部损伤。严重的症状可能会限制运动耐量、需要频繁住院、严重影响生活质量（QOL），并导致早逝。这篇综述文章重点介绍了原发性抗体缺陷患者支气管扩张症的适当识别和管理。基础免疫缺陷和支气管扩张症需要从免疫和肺部的综合角度进行治疗，这在本文中反映了来自这两个领域的专家的观点。积极的多学科方法可能会减少恶化并减缓永久性肺部损伤的进展。
