Bronchiectasis in severe α 1 -antitrypsin deficiency: lessons for the pulmonologist
Abstract
Extract Recent scientific investigation has reshaped the pathophysiological paradigm of bronchiectasis, shifting the focus from chronic infection to chronic inflammation. Central to this inflammatory process is aberrant neutrophil behaviour, particularly the dysregulated activity of neutrophil-derived proteases. Given this mechanistic insight, one might expect deficiency of α1-antitrypsin (α1-AT), a key endogenous protease inhibitor, to be well characterised in this context. However, the relationship between α1-AT deficiency (α1-ATD) and bronchiectasis remains poorly defined.
Fields of Study & Subjects
Cystic Fibrosis Research Advances
Chronic Obstructive Pulmonary Disease (COPD) Research
Protease and Inhibitor Mechanisms
Publication details
- Journal / source
- ERJ Open Research
- Volume
- 12
- Issue
- 1
- Pages
- 01473–2025
- Publication date
- 1 January 2026
- Language
- EN