Vasculitis and Risk of Bronchiectasis in Alpha-1 Antitrypsin Deficiency Subjects from the EARCO Registry

This is the authors' abstract. We don't add key points for this paper.

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].