Inhaled alpha 1-Antitrypsin Administered to Treat Pneumatocele in Autosomal Dominant Hyperimmunoglobulin E Syndrome

Members of the serine protease, matrix metalloproteinase, and cysteinyl protease families have been found to be associated with lung inflammation and airway extracellular matrix [1]. The serine protease neutrophil elastase induces protease and tissue destruction in the lung [1]. Protease-antiprotease imbalance in humans has been described in chronic destructive lung diseases such as cystic fibrosis and alpha -1 antitrypsin deficiency [2,3]. Aerosolized alpha -1 antitrypsin therapy has been administered to restore the protease-antiprotease imbalance and inhibit destructive lung inflammation [4,5].