Treatment of Hereditary Angioedema with a Vapor-Heated C1 Inhibitor Concentrate

Hereditary angioedema, first reported by Quincke in 1882 1 and described by Osler in 1888, 2 is characterized by episodic bouts of well-circumscribed, nonpitting subepithelial edema that primarily involve the extremities, larynx, face, and abdomen. 3 , 4 The condition is inherited as an autosomal dominant trait and characterized by functional levels of C1 inhibitor activity in the blood that are approximately 30 percent of normal values. 5 About 15 percent of patients, however, have normal levels of antigenic C1 inhibitor, but most of it is nonfunctional. 6 As a result of the failure of C1 inhibitor to block the enzymatic activity of C1, levels of . . .