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Bronchiectasis: Current Concepts in Pathogenesis, Immunology, and Microbiology.
Annual Review of Pathology: Mechanisms of Disease ( IF 28.4 ) Pub Date : 2016-03-17 , DOI: 10.1146/annurev-pathol-012615-044344
Rosemary J Boyton 1, 2 , Daniel M Altmann 3
Affiliation  

Bronchiectasis is a disorder of persistent lung inflammation and recurrent infection, defined by a common pathological end point: irreversible bronchial dilatation arrived at through diverse etiologies. This suggests an interplay between immunogenetic susceptibility, immune dysregulation, bacterial infection, and lung damage. The damaged epithelium impairs mucus removal and facilitates bacterial infection with increased cough, sputum production, and airflow obstruction. Lung infection is caused by respiratory bacterial and fungal pathogens, including Pseudomonas aeruginosa, Haemophilus, Aspergillus fumigatus, and nontuberculous mycobacteria. Recent studies have highlighted the relationship between the lung microbiota and microbial-pathogen niches. Disease may result from environments favoring interleukin-17-driven neutrophilia. Bronchiectasis may present in autoimmune disease, as well as conditions of immune dysregulation, such as combined variable immune deficiency, transporter associated with antigen processing-deficiency syndrome, and hyperimmunoglobulin E syndrome. Differences in prevalence across geography and ethnicity implicate an etiological mix of genetics and environment underpinning susceptibility.

中文翻译:

支气管扩张:发病机理,免疫学和微生物学的当前概念。

支气管扩张是一种持续性肺部炎症和反复感染的疾病,其定义为一个常见的病理终点:不可逆的支气管扩张是通过各种病因引起的。这表明免疫遗传易感性,免疫失调,细菌感染和肺损伤之间存在相互作用。受损的上皮损害了粘液的清除,并促进了咳嗽,痰液的产生和气流的阻塞,从而促进了细菌的感染。肺部感染是由呼吸道细菌和真菌病原体引起的,包括铜绿假单胞菌,嗜血杆菌,烟曲霉和非结核分枝杆菌。最近的研究强调了肺微生物群和微生物病原体壁ni之间的关系。疾病可能是由有利于白介素17驱动的嗜中性粒细胞的环境导致的。支气管扩张可存在于自身免疫性疾病中,以及免疫失调的情况下,例如合并的可变免疫缺陷,与抗原加工缺陷综合征相关的转运蛋白和免疫球蛋白E综合征。跨地域和种族的患病率差异暗示着遗传和环境易感性的病因学混合。
更新日期:2019-11-01
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