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Killian Hurley¹, Emer P Reeves¹, Tomás P Carroll¹

¹a Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland Education and Research Centre , Beaumont Hospital , Dublin , Ireland.

Expert Review of Respiratory Medicine

|December 5, 2015

Abstract:

Alpha-1 antitrypsin (AAT) deficiency (AATD) has traditionally been thought of as a genetic disorder characterized by lung destruction and early emphysema in a low AAT, and high neutrophil elastase (NE) environment in the lungs of affected individuals. Recently, a growing body of evidence has emerged to support the hypothesis that tumor necrosis factor alpha (TNF-α) is essential in the pathogenesis of both genetic AATD and non-genetic chronic obstructive pulmonary disease (COPD). Reports have highlighted the importance of TNF-α driven immune cell dysfunction in the development of lung disease in AATD. The authors discuss the role of AAT as a key modulator of TNF-α signaling firstly in the setting of AATD and secondly in other conditions where AAT augmentation therapy has potential utility as a novel therapy.

Keywords:

ADAM-17 Alpha-1 antitrypsin TNF-α alpha-1 antitrypsin augmentation therapy alpha-1 antitrypsin deficiency chronic obstructive pulmonary disease inflammation neutrophils

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