Dept of Pathophysiology and Transplantation, University of Milan, Milan, Italy.
Internal Medicine Dept, Respiratory unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Eur Respir Rev. 2018 Jul 11;27(149). doi: 10.1183/16000617.0016-2018. Print 2018 Sep 30.
Bronchiectasis is a chronic respiratory disease characterised by a syndrome of productive cough and recurrent respiratory infections due to permanent dilatation of the bronchi. Bronchiectasis represents the final common pathway of different disorders, some of which may require specific treatment. Therefore, promptly identifying the aetiology of bronchiectasis is recommended by the European Respiratory Society guidelines. The clinical history and high-resolution computed tomography (HRCT) features can be useful to detect the underlying causes. Despite a strong focus on this aspect of treatment a high proportion of patients remain classified as "idiopathic". Important underlying conditions that are treatable are frequently not identified for prolonged periods of time.The European Respiratory Society guidelines for bronchiectasis recommend a minimal bundle of tests for diagnosing the cause of bronchiectasis, consisting of immunoglobulins, testing for allergic bronchopulmonary aspergillosis and full blood count. Other testing is recommended to be conducted based on the clinical history, radiological features and severity of disease. Therefore it is essential to teach clinicians how to recognise the "clinical phenotypes" of bronchiectasis that require specific testing.This article will present the initial investigation and management of bronchiectasis focussing particularly on the HRCT features and clinical features that allow recognition of specific causes.
支气管扩张症是一种慢性呼吸系统疾病,其特征是由于支气管永久性扩张而导致的咳嗽和反复呼吸道感染综合征。支气管扩张症是不同疾病的最终共同途径,其中一些疾病可能需要特定的治疗。因此,欧洲呼吸学会指南建议及时确定支气管扩张症的病因。临床病史和高分辨率计算机断层扫描(HRCT)特征有助于发现潜在病因。尽管治疗的重点在这一方面,但仍有很大一部分患者被归类为“特发性”。重要的潜在可治疗的疾病在很长一段时间内都未被发现。欧洲呼吸学会支气管扩张症指南建议进行一套最低限度的检测来诊断支气管扩张症的病因,包括免疫球蛋白、变应性支气管肺曲霉病检测和全血细胞计数。其他检测建议根据临床病史、影像学特征和疾病严重程度进行。因此,教会临床医生如何识别需要特定检测的支气管扩张症的“临床表型”至关重要。本文将重点介绍支气管扩张症的初步检查和管理,特别关注允许识别特定病因的 HRCT 特征和临床特征。