Bennett William D, Henderson Ashley G, Donaldson Scott H
1 Pulmonary and Critical Care Medicine, Department of Medicine.
2 Marsico Lung Institute, Cystic Fibrosis Research Center, and.
Ann Am Thorac Soc. 2016 Apr;13 Suppl 2(Suppl 2):S186-90. doi: 10.1513/AnnalsATS.201509-652KV.
Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis. A deeper understanding of how tobacco smoke and other triggers of chronic bronchitis actually lead to disease, and exploration of the concept that therapies that restore cystic fibrosis transmembrane conductance regulator function, mucus hydration, and/or mucociliary clearance may benefit patients with chronic bronchitis, hold the prospect of significant progress in treating this prevalent disease.
患有慢性阻塞性肺疾病的慢性支气管炎型和囊性纤维化的患者具有相似的临床特征,包括气道黏液阻塞以及慢性/复发性气道感染的发生,这些感染常表现为疾病加重。越来越多的证据表明,这些疾病在发病机制上也可能存在相似之处,包括囊性纤维化跨膜传导调节因子功能障碍、黏液脱水以及黏液纤毛清除功能缺陷。随着针对导致囊性纤维化肺病的基本缺陷的治疗方法不断取得进展,类似的方法也有可能使慢性支气管炎患者受益。更深入地了解烟草烟雾和慢性支气管炎的其他触发因素如何实际导致疾病,并探索恢复囊性纤维化跨膜传导调节因子功能、黏液水合作用和/或黏液纤毛清除功能的疗法可能使慢性支气管炎患者受益的概念,有望在治疗这种常见疾病方面取得重大进展。