Vasculitis and risk of bronchiectasis in alpha-1 antitrypsin deficiency subjects from the EARCO registry
Open Access
- 1 October 2026
- journal article
- Published by European Respiratory Society (ERS) in European Respiratory Journal
- Vol. 68 (5) , 2600779
Abstract
Extract Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder caused by variants in the SERPINA1 gene, resulting in reduced circulating AAT levels [1]. Deficiency of functional AAT disrupts the protease antiprotease balance and predisposes individuals to early-onset pulmonary emphysema[2]. While liver disease is driven by intracellular accumulation of misfolded and polymerised Z-AAT within hepatocytes[1, 3].Keywords
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