Pathogenesis of Bronchiectasis

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Cystic fibrosis (CF) typically follows a more severe clinical course than non-CF bronchiectasis. Despite this recognized difference, the underpinnings of respiratory biology support a common pathogeneses of the anatomic deformations of bronchiectasis. This article reviews the observed manifestations among the related diseases of bronchiectasis and CF and discusses some of their similarities and differences. As more details of the mechanisms of bronchiectasis are unveiled, more parallels among the seemingly disparate causes of CF and non-CF bronchiectasis are recognized. With these insights, more opportunities to halt the vicious circle have become apparent.

Section snippets

Cystic fibrosis: pathogenesis of bronchiectasis

Despite improvements in childhood health—nutrition, immunizations, tuberculosis control, and improved access to antibiotics—chronic pulmonary infections and bronchiectasis remain significant clinical issues worldwide. Bronchiectasis manifests differently among patients, particularly in the areas of inciting pathology, causative organisms, and prognosis. Specifically, cystic fibrosis (CF) typically follows a more severe clinical course than non-CF bronchiectasis. Despite these and other

Hygiene disruption in the cystic fibrosis airway

In the normal human respiratory tract, several components of innate immunity protect the respiratory tract from constant environmental exposures (particulates, microorganisms, oxygen, and so forth). Fundamental among these protections within the lower respiratory tract are airway clearance (primarily the mucociliary elevator), physicochemical barriers (represented by the mucus-sol constituents of the airway surface liquid [ASL]), and cell-based innate defenses.

Summary

Outlined in this article are the primary and acquired abnormalities of the CF airway that stimulate the genesis of and promulgate the pathology of bronchiectasis. Inflammation and infection are central causative mechanisms of bronchiectasis. Their persistence appears to disrupt orderly reparative processes in the airway. Modulation of these processes and controlling the positive feedback of bronchiectasis afford some braking effects on the inexorable respiratory decline in patients who have CF

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