C1 esterase inhibitor concentrate

General information

Hereditary angioedema, which is characterized by episodic bouts of swelling of submucosal and subcutaneous tissue, is due to a hereditary abnormality of C1 esterase inhibitor, either a deficiency of the normal enzyme or more rarely the presence of an abnormal enzyme. If C1 esterase inhibitor deficiency can be demonstrated, infusion of concentrated C1 esterase inhibitor is effective in treating the attack. In three studies, no adverse effects were recorded [ ].

Hereditary angioedema, which is effectively and safely treatable with C1 esterase inhibitor concentrates, continues to constitute a significant burden of disease to those affected even in the industrialized world. Other therapeutic indications for C1 esterase inhibitor concentrates relating to inflammatory disease other than are currently being studied, including post-ischemic myocardial protection, capillary leak syndrome, and sepsis [ , ]. The main adverse effects of C1 esterase inhibitor concentrate, although very rare, are allergic reactions. In a study of the beneficial effects of C1 esterase inhibitor concentrates in ST-elevation myocardial infarction in patients undergoing surgical reperfusion, no adverse events were reported [ ].