ERS指南翻译

Exacerbations of bronchiectasis are key targets for therapy as they are major determinants of healthcare costs. They are associated with increased airways and systemic inflammation and progressive lung damage . In addition, more severe and more frequent exacerbations are associated with worse quality of life, daily symptoms , lung function decline, and mortality. Consequently, the majority of therapeutic interventions are aimed at reducing exacerbations. Despite current treatment approaches, European registry data shows that approximately 50% of European bronchiectasis patients have two or more exacerbations per year and one third require at least one hospitalisation per year .支气管扩张的恶化是治疗的关键目标，因为它们是医疗费用的主要决定因素。

它们与呼吸道的增加和全身炎症和肺损伤有关。

此外，更严重、更频繁的恶化与生活质量、日常症状、肺功能减退和死亡率有关。

因此，大多数的治疗干预措施都是为了减少恶化。

尽管目前的治疗方法，欧洲注册中心的数据显示，大约50%的欧洲支气管扩张患者每年有两种或更多的恶化，三分之一的患者每年至少需要一次住院治疗。

Our understanding of what causes symptoms and exacerbations is based on the vicious cycle concept, with key components of the disease being chronic bronchial infection, inflammation, impaired mucociliary clearance and structural lung damage. Treatment is primarily based on the principles of preventing or suppressing acute and chronic bronchial infection, improving mucociliary clearance and reducing the impact of structural lung disease. 我们对导致症状和恶化的原因的理解是基于恶性循环的概念，它的主要成分是慢性支气管感染、炎症、粘膜纤毛清除和结构性肺损伤。

治疗主要基于预防或抑制急性和慢性支气管感染的原则，改善粘膜纤毛间隙并减少结构性肺部疾病的影响。

Chronic airways infection, most frequently with Haemophilus influenzae and Pseudomonas aeruginosa and less frequently with Moraxella catarrhalis, Staphylococcus aureus and Enterobacteriaceae, stimulate and sustain lung inflammation. Persistent isolation of these organisms in sputum or bronchoalveolar lavage is associated with an increased frequency of exacerbations, worse quality of life and increased mortality. This is particularly the case with P. aeruginosa infection. A systematic review of observational studies identified that P. aeruginosa infection is associated with a three-fold increase in mortality risk, an almost seven-fold increase in risk of hospital admission and an average of one additional exacerbation per patient per year .慢性呼吸道感染，最常见的是流感病毒和Pseudomonas的流感病毒，以及更少的Moraxella白内障、金黄色葡萄球菌和肠杆菌科，刺激和维持肺部炎症。

这些微生物在痰或支气管肺泡内的持续隔离与增加的恶化频率、生活质量的恶化和死亡率的增加有关。

这是一种特殊的情况，有p.aeruginosa感染。

一项系统性的观察性研究发现，暴露于空气中感染的人的死亡风险增加了三倍，住院的风险增加了近7倍，平均每名患者每年增加一次。

Mucociliary clearance is impaired by the impact of structural bronchiectasis, airway dehydration, excess mucus volume and viscosity. More than 70% of bronchiectasis patients expectorate sputum daily with highly variable sputum volumes. Treatment aims to prevent mucus stasis and the associated mucus plugging, airflow obstruction and progressive lung damage.由于结构支气管扩张、气道脱水、粘液体积过多和粘度的影响，粘膜纤毛的清除会受到损害。

超过70%的支气管扩张患者每天痰液量大，痰量大。

治疗的目的是防止粘液阻塞和相关的粘液堵塞，气流阻塞和渐进性肺损伤。

Structural changes in the lung associated with disease include bronchial dilatation, bronchial wall thickening, and mucus plugging as well as small airways disease and emphysema. More than 50% of patients have airflow obstruction, but restrictive, mixed ventilatory pattern and preserved lung function are also frequently observed. Breathlessness is due to the impact of airflow obstruction, impaired gas transfer, exercise deconditioning and the impact of comorbidities. Breathlessness is one of the strongest predictors of mortality. Therapies may aim totreat airflow obstruction (e.g. bronchodilators), to improve exercise capacity (pulmonary rehabilitation), or to remove poorly functioning or diseased lung(e.g. surgery).与疾病相关的肺部结构变化包括支气管扩张、支气管壁增厚、粘液堵塞以及小呼吸道疾病和肺气肿。

超过50%的患者有气流障碍，但也经常观察到限制性的、混合的通风模式和肺功能。

呼吸困难是由于气流受阻、气体转移受损、运动减少和共病的影响造成的。

呼吸困难是死亡的最有力的预测因素之一。

治疗的目的可能是治疗气流障碍(如支气管扩张器)，改善运动能力(肺部康复)，或消除功能低下或患病的肺部(如:手术)。

Bronchiectasis has long been a neglected disease. The prevalence of bronchiectasis has been estimated at 53 to 566 cases per 100 000 inhabitants. Prevalence increases with age and female gender .长期以来，支气管扩张一直是一种被忽视的疾病。

据估计，每10万居民中，支气管扩张的发生率为53至566例。

患病率随着年龄和女性的性别而增加。

QUINT et al. described that age-adjusted mortality rate for bronchiectasis was 1437.7 per 100 000. Several longitudinal studies have described up to a 30% mortality at 1-year follow-up after suffering an exacerbation, particularly in the presence of COPD.QUINT等人描述了支气管扩张的年龄调整死亡率为每10万人1437.7。

几项纵向研究表明，在经历了一种恶化之后，在1年的随访中死亡率为30%，尤其是在COPD患者出现的情况下。

The economic burden of this disease has been estimated to be similar to COPD; this increases with disease severity, hospitalisations, need for intensive care, and use of inhaled antibiotics. No therapies are currently specifically licensed by regulatory authorities in Europe or the USA for the treatment of bronchiectasis. Historically, treatment has been extrapolated from the management of cystic fibrosis bronchiectasis, but randomised clinical trials and clinical experience has demonstrated that treatment responses are different and that specific guidance for bronchiectasis not due to cystic fibrosis is necessary.据估计，这种疾病的经济负担与COPD相似;这增加了疾病的严重性，住院治疗，需要重症监护，以及使用吸入的抗生素。