Department of Translational Medical Sciences, Section of Paediatrics, Federico II University, Naples, Italy.
Pulmonary Disease and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Respirology. 2019 Nov;24(11):1063-1072. doi: 10.1111/resp.13615. Epub 2019 Jun 20.
Bronchiectasis, conventionally defined as irreversible dilatation of the bronchial tree, is generally suspected on a clinical basis and confirmed by means of chest high-resolution computed tomography. Clinical manifestations, including chronic productive cough and endobronchial suppuration with persistent chest infection and inflammation, may deeply affect quality of life, both in children/adolescents and adults. Despite many cases being idiopathic or post-infectious, a number of specific aetiologies have been traditionally associated with bronchiectasis, such as cystic fibrosis (CF), primary ciliary dyskinesia or immunodeficiencies. Nevertheless, bronchiectasis may also develop in patients with bronchial asthma; chronic obstructive pulmonary disease; and, less commonly, rheumatological disorders and inflammatory bowel diseases. Available literature on the development of bronchiectasis in these conditions and on its management is limited, particularly in children. However, bronchiectasis may complicate the clinical course of the underlying condition at any age, and appropriate management requires an integration of multiple skills in a team of complementary experts to provide the most appropriate care to affected children and adolescents. The present review aims at summarizing the current knowledge and available evidence on the management of bronchiectasis in the other conditions mentioned and focuses on the new therapeutic strategies that are emerging as promising tools for improving patients' quality of life.
支气管扩张症通常定义为支气管树的不可逆扩张,临床上主要根据临床表现怀疑,并通过胸部高分辨率计算机断层扫描来确诊。临床表现包括慢性咳痰和支气管内化脓性感染以及持续性炎症,这些症状可能会严重影响儿童/青少年和成年人的生活质量。尽管许多病例是特发性或感染后,但传统上一些特定病因与支气管扩张症有关,例如囊性纤维化(CF)、原发性纤毛运动障碍或免疫缺陷。然而,支气管扩张症也可能发生在支气管哮喘、慢性阻塞性肺疾病的患者中;较少见的是,风湿性疾病和炎症性肠病。关于这些情况下支气管扩张症的发展及其管理的可用文献有限,特别是在儿童中。然而,支气管扩张症可能会在任何年龄使基础疾病的临床病程复杂化,适当的管理需要多学科团队的综合技能,以提供最适合受影响的儿童和青少年的护理。本综述旨在总结目前关于支气管扩张症在上述其他疾病中的管理的知识和现有证据,并侧重于新的治疗策略,这些策略作为改善患者生活质量的有前途的工具正在不断涌现。