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Immune regulation in idiopathic bronchiectasis
Author(s) -
Boyton Rosemary J.,
Altmann Daniel M.
Publication year - 2012
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2012.06756.x
Subject(s) - bronchiectasis , immunology , medicine , immune system , immune dysregulation , pathogenesis , common variable immunodeficiency , disease , acquired immune system , lung , pathology , antibody
Bronchiectasis is a complex pathological endpoint arrived at through a diverse interplay between lung infection and altered immune function. It comprises irreversible, abnormal dilatation of one or more bronchi, with chronic airway inflammation and is associated with recurrent chest infections, airflow obstruction, chronic cough, excessive sputum production, and malaise. Many pathogens are associated with this disease, including chronic bacterial infections, nontuberculous mycobacteria, and aspergillis. However, the etiology is poorly defined. Disease‐associated genes indicate a likely contribution to disease mechanism both from innate and adaptive immunity. The role of immune mechanisms is highlighted by the occurrence of bronchiectasis in a subset of patients with rheumatoid arthritis or inflammatory bowel disease as well as diseases of immune dysregulation such as combined variable immune deficiency, transporter associated with antigen processing (TAP) deficiency syndrome, and hyper‐IgE syndrome. Recent evidence indicates a possible role of excessive natural killer cell activation in pathogenesis.