◀ Prev · Parts · Full · Next ▶

Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema.cite-ref-overview-of-complement-1-5[1] C1-inhibitor defiency can be hereditary or acquired, resulting in hereditary or acquired angioedema.cite-ref-angiodema-12-0[12] C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity.cite-ref-13[13]cite-ref-angiodema-12-1[12] C1-inhibitor controls the processes involved in maintaining vascular permeability. As a result, C1-inhibitor levels of less than 50% of the standard lead to increased vascular permeability, characteristic of angioedema.cite-ref-angiodema-12-2[12] Cinryze, a human plasma derived C1-esterase inhibitor, has been approved for use in 2008 for the prevention of hereditary angioedema attacks.cite-ref-14[14]cite-ref-15[15]

◀ Prev · Parts · Full · Next ▶