What is Cinryze used for and how does it work?

• Cinryze is given to prevent angioedema attacks in adults and adolescents with hereditary angioedema (HAE).
• Cinryze is a man-made form of a protein called C1 esterase inhibitor, that naturally occurs in our blood.
• People with HAE have reduced levels of this protein in their blood, which leads to attacks of severe swelling in the limbs, face, intestinal tract, and airway.
• Cinryze replaces missing C1 protein in the blood of people with HAE, preventing the generation of bradykinin which is a protein fragment responsible for increasing the permeability of blood vessels.

Cinryze is a brand of complement C1 esterase inhibitor (C1 inhibitor). C1 inhibitor is a man-made form of a protein in blood that helps control swelling in the body and is used to treat people with a condition called hereditary angioedema (HAE) who lack the C1 protein in their blood.

Hereditary angioedema is a genetic disorder that causes attacks of severe swelling in the limbs, face, intestinal tract, and airway.

What is Cinryze?

Cinryze (complement C1 esterase inhibitor) is a man-made form of the C1 inhibitor protein that naturally occurs in the blood.

The main purpose of C1 inhibitor protein is to regulate the coagulation pathway (the process that causes our blood to clot), the fibrinolytic system (the process that removes and degrades clots after blood vessels are repaired), and to control the production of bradykinin, which is a protein fragment that increases the movement of fluid through blood vessel walls, resulting in swelling and inflammation.

How does Cinryze work?

People with hereditary angioedema (HAE) have low levels of naturally occurring C1 protein in their blood. Cinryze increases levels of C1 protein, which reduces bradykinin production, decreasing the permeability of blood vessels and reducing swelling.

Cinryze is used to prevent attacks of angioedema in people with HAE.

How is Cinryze given?

Cinryze is given by slow injection every three or four days to prevent angioedema attacks in people with HAE.

Are there any other types of C1 inhibitors?

Yes, there are three other types of C1 inhibitors:

• Berinert, which is used to treat attacks of angioedema
• Ruconest, which is used to treat attacks of angioedema
• Haegarda, which is given every three to four days to prevent attacks of angioedema.

What is hereditary angioedema (HAE)?

Hereditary angioedema is a genetic disorder characterized by severe swelling in the limbs, face, intestinal tract, and airway. There are at least three known types: Type I and II caused by mutations in the Serping I gene and Type III which is caused by mutations in the F12 gene.

The Serping I gene provides instructions for making the C1 inhibitor protein, which helps control inflammation. Mutations in this gene can lead to either reduced levels of C1 protein in the blood or the production of a C1 inhibitor that functions abnormally. When levels of this C1 protein are decreased, excessive amounts of another protein fragment, called bradykinin are generated. Bradykinin increases the leakage of fluid through the walls of blood vessels into body tissues, promoting inflammation. This causes fluid to accumulate which causes the swelling characteristic of hereditary angioedema type I and type II.

Some cases of hereditary angioedema type III are associated with mutations in the F12 gene. This gene codes for an important protein that helps our blood to clot, known as coagulation factor XII. Factor XII also stimulates inflammation and is involved in bradykinin production. Certain F12 mutations produce Factor XII with an increased activity, which generates more bradykinin, leading to blood vessel wall leakage and angioedema. The gene mutations responsible for other types of hereditary angioedema III have not yet been discovered.