Clinical Effect of Alpha-1 Antitrypsin Deficiency in Antineutrophil Cytoplasmic Antibody-associated Vasculitis: Results from a French Retrospective Monocentric Cohort.
Samuel DeshayesNicolas Martin SilvaFrédérique GrandhommeKathy KhoyDelphine MariotteJonathan BoutemyGwénola MaignéClaire Brière-BellierClaire DelmasBoris BienvenuThierry LobbedezHubert de BoyssonAchille AoubaPublished in: The Journal of rheumatology (2019)
This study shows that AAT deficiency confers, independently of ANCA subtype, a higher risk of IAH. Prospective studies are required to refine these data and to assess the need for replacement therapy in AAT-deficient patients with AAV.