The Role of C1-INH in Other Diseases

The Complement System works in conjunction with other regulatory cascades within the body including the Contact and Coagulation Systems to maintain a normal state of health. A key regulator of these systems is C1 esterase inhibitor (C1-INH). These systems are controlled through a series of tightly controlled feedback loops which allow interaction between these systems. The aggregated response of these systems results in an internal equilibrium, allowing the body to respond to an insult while minimizing harm done to healthy cells.

Imbalance in the activation and regulation of the Complement system has been associated with a variety of disease states including hereditary angioedema (HAE), antibody-mediated rejection in transplant recipients, macular degeneration, infectious diseases including community-acquired pneumonia (CAP), neurologic/neurodegenerative conditions such as Alzheimer’s disease, ischemia/reperfusion injury including acute myocardial infarction (AMI), and a variety of inflammatory conditions including rheumatoid arthritis, systemic lupus erythematosis and inflammatory bowel disease (IBD).