How do we treat patients with hereditary angioedema

Hereditary angioedema (HAE) is due to the inherited deficiency of C1-Inhibitor (C1-Inh). When specific treatment was not available, the mortality rate for this disease was as high as 50% and the disability up to 100-150 days per year [Agostoni and Cicardi, Hereditary and acquired Cl-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore) 71 (1992) 206]. Stich a worrying scenario dramatically improves upon appropriate treatment. Nevertheless, the disease still frequently goes undiagnosed or misdiagnosed as an allergic condition. Both circumstances prevent patients from receiving drugs that could save and/or improve the quality of their life. The interest of our group for patients with HAE goes back to the early seventies [Agostoni and Martignoni, Hereditary angioneurotic oedema. Lancet 2 (1973) 325]. Since that time, 441 such patients have been examined and treated at our department, 403 are still actively followed. Here we present our experience oil the treatment of HAE. (C) 2003 Elsevier Ltd. All rights reserved.