Advances in Hereditary Angioedema: The Prevention of Angioedema Attacks With Subcutaneous C1-Inhibitor Replacement

William Lumry1,2,3,4,5, Teri Templeton1,2,3,4,5, Laurel Omert1,2,3,4,5

  • 1AARA Research Center, Dallas, Texas (Dr Lumry); US HAEA Angioedema Center at University of California San Diego, San Diego, California (Ms Templeton); CSL Behring, King of Prussia, Pennsylvania (Dr Omert); University of California Irvine School of Medicine, Orange, California (Dr Levy).

Hereditary angioedema (HAE) is a debilitating condition caused by a functional C1-inhibitor (C1-INH) deficiency and characterized clinically by episodes of subcutaneous or submucosal swelling. C1-INH replacement is highly effective for preventing HAE attacks and can improve health-related quality of life. Once available only for intravenous use, C1-INH is now available as a subcutaneous formulation for self-administration, shown to provide sustained plasma levels of C1-INH and reducing the monthly median HAE attack rate by 95% versus placebo in the phase 3 COMPACT study. Subcutaneously administered C1-INH satisfies multiple unmet needs in the management of patients with HAE.

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