遗传性血管水肿
缓激肽
医学
血管性水肿
内科学
单克隆抗体
皮肤病科
胃肠病学
免疫学
抗体
受体
作者
João Teixeira,Duarte Flor,Joana Xará,Margarida Gonçalo
摘要
Type I hereditary angioedema (HAE) is a rare autosomal dominant disorder characterized by recurrent bradykinin-mediated angioedema affecting the skin, respiratory, and gastrointestinal tracts. Lanadelumab, a monoclonal antibody targeting plasma kallikrein to reduce bradykinin production, has shown efficacy in long-term prophylaxis (LTP) for patients aged 2 and above, with a recommended dose of 300 mg biweekly. We present two cases of type I HAE patients, poorly controlled with traditional LTP therapies, demonstrating complete response and extended attack-free intervals with lanadelumab, suggesting that administration intervals beyond 4 weeks may be feasible, improving patient quality of life and healthcare cost-effectiveness.
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