Therapeutic management of hereditary angioedema: past, present, and future

Abstract

Hereditary angioedema is a rare disease that can often be disablingor even life threatening because of the unpredictable, self-limiting, and localized swelling episodes involving cutaneous, subcutaneous, and mucosal sites. The last decades revealed a spectrumof possibilities to control the disease through the development ofeffective therapies that changed the life of many patients and families worldwide.This review summarizes the current literature regarding the generalmanagement and therapeutic approach in patients with hereditary angioedema, both with and without C1 inhibitor deficiency. Medicationsalready available in the market and new drugs in different research stages of development are addressed.Recent decades saw a huge leap in identifying mechanisms of angioedema and developing modern safe and effective medications to both treatacute angioedema manifestations and control disease activity via prophylactic therapy. Further improvement is still needed, together with improving global accessibility of diagnostic tools and effective medications.Whether novel drugs will demonstrate a sustained cost/effectiveness ratiowill be answered in the years to come when we will witness whether amajority of the patients will benefit from these major advances.