遗传性血管性水肿治疗

Therapy of Hereditary Angioedema

遗传性血管性水肿(hereditary angioedema,HAE)是一种较为罕见的常染色体显性遗传病,临床上表现为反复发生的皮肤和黏膜水肿,当水肿发生于呼吸道时可窒息死亡。由于该病发病率低,临床医生对其认识不足常被误诊、误治。随着对该病研究的不断深入,这一疾病逐渐被人们认识并重视。尤其是对HAE发病机制的研究,使得多种新药被研制出并已应用于临床。本文对该病目前国内外现行的治疗方法进行综述,以期帮助临床医生正确选择治疗方案。

Hereditary angioedema （HAE） is a rare autosomal dominant inherited disease, which was used to be misdiagnosed. It is a characterized by recurrent subcutaneous and submucous edema. It can be life-threatening when the edema occurres in the upper airway. New therapeutic methods are emerging based on the progress in the field of pathogenesis of HAE. Current opinions on therapy of HAE are summarized in this review.