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Alpha-1-proteinase inhibitor is a purified human plasma-derived serine protease inhibitor (serpin) used as augmentation therapy in adults with emphysema due to congenital deficiency of the protein. Its primary mechanism is inhibition of neutrophil elastase and other serine proteases in the lungs. By preventing excessive elastolytic activity and tissue destruction in alveolar structures caused by unopposed neutrophil elastase activity—especially in patients with genetic deficiency—it helps slow progression of emphysema. The drug also has immunomodulatory effects on both innate and adaptive immune cells. It is prepared from pooled human plasma using fractionation and purification processes[2][3][5][6].
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