Evidence‐based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group

TLDR

Hereditary C1 inhibitor deficiency causes rare, life‑threatening angioedema, and recent trials have introduced new therapies, yet existing guidelines remain outdated and based largely on observational data. The authors convened a conference to develop evidence‑based treatment guidelines for HAE. The conference brought together 58 experts, industry representatives, and patient advocates to discuss HAE management. The consensus identified evidence‑based management strategies for adult and adolescent HAE patients. © 2012 Allergy 67:147–157; Cicardi et al.

Abstract

To cite this article: Cicardi M, Bork K, Caballero T, Craig T, Li HH, Longhurst H, Reshef A, Zuraw B on behalf of HAWK ( H ereditary A ngioedema International W or k ing Group). Evidence‐based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy 2012; 67 : 147–157. Abstract Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life‐threatening, disabling disease. In the last 2 years, the results of well‐designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence‐based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010. The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical companies and representatives of HAE patients’ associations. Here, we report the topics discussed during the meeting and evidence‐based consensus about management approaches for HAE in adult/adolescent patients.