SERPINA1 and alpha 1-antitrypsin deficiency: Further work demonstrated that augmentation therapy restored AAT plasma levels and normalized TNF-α signaling, thereby preventing TNF-α-induced neutrophil release of secondary and tertiary granules and resultant production of autoantibodies.80 Additionally, it was recently reported that AATD is associated with increased neutrophil membrane-bound NE, which can trigger an inflammatory cycle inducing secretion of LTB4 that further stimulates primary granule release.