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Annu Rev Pathol. 2016 May 23;11:523-54. doi: 10.1146/annurev-pathol-012615-044344. Epub 2016 Mar 2.

Bronchiectasis: Current Concepts in Pathogenesis, Immunology, and Microbiology.

Author information

1
Lung Immunology Group, Department of Medicine, Imperial College London, London W12 0NN, United Kingdom; email: r.boyton@imperial.ac.uk.
2
Department of Respiratory Medicine, Royal Brompton & Harefield NHS Foundation Trust, London SW3 6NP, United Kingdom.
3
Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London W12 0NN, United Kingdom.

Abstract

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.

KEYWORDS:

bacterial infection; immunity; lung; microbiota

[Indexed for MEDLINE]

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