Rediscovery of a forgotten disease: Hereditary Angioedema

Abstract

HAE is a rare hereditary disease that causes recurrent angioedema attacks. Attacks are often disturbing. However, it can sometimes cause death due to laryngeal edema. The disease is often not considered, and therefore the diagnosis of patients is often delayed. Although it was first described in the 19th century, pathogenesis was understood at the end of the 20th century. With the understanding of its pathogenesis, improvements in the diagnosis and treatment of the disease in the last 20 years led to a significant improvement in patients' prognosis, and quality of life.