Yap Vanessa L, Metersky Mark L
Division of Pulmonary and Critical Medicine, University of Connecticut School of Medicine, Farmington, Connecticut, USA.
Curr Opin Infect Dis. 2015 Apr;28(2):171-6. doi: 10.1097/QCO.0000000000000147.
Patients with noncystic fibrosis bronchiectasis (NCFB) share many of the respiratory symptoms of cystic fibrosis and often are provided therapies effective in cystic fibrosis, often without clear evidence of benefit. There are currently no approved therapies for NCFB, but in recent years, there has been increased interest in developing new therapies due to the increasing prevalence and perceived unmet needs. This review is meant to provide the most recent information to clinicians about currently available and pipeline therapies for NCFB.
Inhaled antibiotics may provide effective bacterial suppressive therapy with an acceptable safety profile in adults with NCFB, although evidence of improved outcomes is limited. Inhaled hyperosmolar agents such as hypertonic saline and mannitol are promising but study results have been mixed. Macrolide antibiotics have anti-inflammatory properties and, in several randomized controlled trials, demonstrated the benefit of chronic low-dose treatment. Other anti-inflammatory agents that have shown promising preliminary results include statins and neutrophil elastase inhibitors.
There is high-quality evidence supporting chronic low-dose macrolide therapy in patients with NCFB. There is limited evidence of benefit of other therapies, including inhaled antibiotics and pharmacologic agents to enhance mucus clearance.
非囊性纤维化支气管扩张症（NCFB）患者具有许多囊性纤维化的呼吸道症状，且常常接受对囊性纤维化有效的治疗，而往往没有明确的获益证据。目前尚无获批用于NCFB的疗法，但近年来，由于患病率上升以及存在未满足的需求，开发新疗法的兴趣日益浓厚。本综述旨在为临床医生提供有关NCFB目前可用疗法和在研疗法的最新信息。
吸入性抗生素可能为患有NCFB的成人提供有效的细菌抑制疗法，且安全性可接受，尽管改善结局的证据有限。吸入性高渗剂，如高渗盐水和甘露醇，前景良好，但研究结果不一。大环内酯类抗生素具有抗炎特性，在多项随机对照试验中，证明了长期低剂量治疗的益处。其他显示出有前景的初步结果的抗炎药包括他汀类药物和中性粒细胞弹性蛋白酶抑制剂。
有高质量证据支持对NCFB患者进行长期低剂量大环内酯类治疗。其他疗法（包括吸入性抗生素和增强黏液清除的药物）的获益证据有限。
