Scottish Centre for Respiratory Research, University of Dundee, Ninewells Hospital and Medical School, Dundee, UK.
Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, Northern Territory, Australia.
Nat Rev Dis Primers. 2018 Nov 15;4(1):45. doi: 10.1038/s41572-018-0042-3.
Bronchiectasis refers to abnormal dilatation of the bronchi. Airway dilatation can lead to failure of mucus clearance and increased risk of infection. Pathophysiological mechanisms of bronchiectasis include persistent bacterial infections, dysregulated immune responses, impaired mucociliary clearance and airway obstruction. These mechanisms can interact and self-perpetuate, leading over time to impaired lung function. Patients commonly present with productive cough and recurrent chest infections, and the diagnosis of bronchiectasis is based on clinical symptoms and radiological findings. Bronchiectasis can be the result of several different underlying disorders, and identifying the aetiology is crucial to guide management. Treatment is directed at reducing the frequency of exacerbations, improving quality of life and preventing disease progression. Although no therapy is licensed for bronchiectasis by regulatory agencies, evidence supports the effectiveness of airway clearance techniques, antibiotics and mucolytic agents, such as inhaled isotonic or hypertonic saline, in some patients. Bronchiectasis is a disabling disease with an increasing prevalence and can affect individuals of any age. A major challenge is the application of emerging phenotyping and endotyping techniques to identify the patient populations who would most benefit from a specific treatment, with the goal of better targeting existing and emerging treatments and achieving better outcomes.
支气管扩张是指支气管的异常扩张。气道扩张可导致黏液清除失败和感染风险增加。支气管扩张的病理生理机制包括持续的细菌感染、免疫反应失调、黏液纤毛清除功能受损和气道阻塞。这些机制可以相互作用和自我延续,导致肺功能逐渐受损。患者常表现为咳痰和反复肺部感染,支气管扩张的诊断基于临床症状和影像学发现。支气管扩张可能是几种不同潜在疾病的结果,确定病因对于指导治疗至关重要。治疗旨在减少恶化的频率,提高生活质量并预防疾病进展。尽管监管机构尚未批准任何药物专门用于治疗支气管扩张,但有证据表明气道清除技术、抗生素和黏液溶解剂(如吸入等渗或高渗盐水)对某些患者有效。支气管扩张是一种致残性疾病,其患病率不断增加,可影响任何年龄段的个体。一个主要挑战是应用新兴的表型和内型技术来确定最受益于特定治疗的患者人群,目的是更好地针对现有和新兴治疗方法并取得更好的结果。