Redondo Margarida, Keyt Holly, Dhar Raja, Chalmers James D
Pulmonology Dept, Centro Hospitalar de Sao Joao, Porto, Portugal.
University of Texas Health Science Center at San Antonio, San Antonio, TX, USA.
Breathe (Sheff). 2016 Sep;12(3):222-235. doi: 10.1183/20734735.007516.
To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis.To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.
Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF), an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different "bronchiectasis syndromes". The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.
认识与支气管扩张相关疾病谱的临床和影像学表现。理解不同全球医疗体系中支气管扩张和囊性纤维化在病因、微生物学及疾病负担方面的差异。
支气管扩张是指支气管扩张,通常为永久性扩张,并伴有咳嗽、咳痰及反复呼吸道感染的临床综合征。它可由一系列遗传性和获得性疾病引起,也可能本质上是特发性的。在西方国家,最广为人知的遗传性疾病是囊性纤维化(CF),这是一种常染色体隐性疾病,可导致进行性支气管扩张、细菌感染和过早死亡。由CF引起的支气管扩张和由其他病症引起的支气管扩张在国际上对医疗体系造成的负担都日益加重。CF的治疗正变得越来越有效,导致更多有复杂医疗需求的成年患者。非CF引起的支气管扩张越来越受到重视,尤其是在老年人群中。识别很重要,可导致找出潜在病因、进行适当治疗并改善生活质量。该疾病的表现形式高度多样,要求所有呼吸内科医生了解不同的“支气管扩张综合征”。最常见的病因和表现综合征因地域而异,非结核分枝杆菌病在北美某些地区占主导,感染后和特发性疾病在西欧占主导,而结核后支气管扩张在南亚和东欧占主导。正在进行的全球合作研究将极大地推进我们对支气管扩张和CF国际影响的理解。