Journal article Open Access
S.Ghani , I.Errabih , N. Benzzoubeir , F.Souidine and L. Ouazzani.
Hereditary angioedema (HAE) is part of the bradykininangioedema. It is a rare and life-threatening disease that affects about 1 in 10,000 to 50,000 people. It is a genetic disease; associated with an abnormality on the C1-inhibitor gene that leads to a deficiency of C1-inhibitor (INH), this deficiency results in uncontrolled activation of the contact system of coagulation and the classical complement pathway, resulting in excessive production of bradykinin. This disease is manifested by acute attacks of intermittent cutaneous or mucosal edema. The angioedema of the digestive tract or abdominal viscera causes intense pain, often accompanied by vomiting related to edematous intestinal obstruction, these edemas usually evolve in a few hours and persist for a few days. These crises are unpredictable and can be very serious if they affect the respiratory sphere. In this \\\"development\\\", after a brief review of the pathophysiology of angioedema (AE), we will detail the clinical manifestations, diagnosis and management of HAE, with a particular focus on new therapeutic agents and treatment modalities.