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Alpha1-proteinase inhibitor is a plasma-derived sterile preparation of purified human alpha1-proteinase inhibitor (A1PI), also known as alpha1-antitrypsin. It is indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of A1PI. As a serine protease inhibitor (serpin), its primary therapeutic mechanism involves the inhibition of neutrophil elastase, an enzyme capable of degrading lung alveolar tissue. In individuals with A1PI deficiency, an imbalance between the protease and the inhibitor leads to progressive destruction of the lung parenchyma. Augmentation therapy with this drug increases and maintains antigenic and functional levels of A1PI in the epithelial lining fluid of the lungs, thereby slowing the progression of emphysema. It is typically administered via weekly intravenous infusion.
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