Lock R J, Gompels M M
Immunology and Immunogenetics, North Bristol NHS Trust, Southmead Hospital, Westbury-on-Trym, Bristol, BS10 5NB, UK.
Curr Allergy Asthma Rep. 2007 Jul;7(4):264-9. doi: 10.1007/s11882-007-0039-6.
Hereditary angioedema, an autosomal dominant disorder, presents clinically as recurrent episodes of swelling. It results from either deficient production or function of C1 inhibitor. Acquired angioedema is associated with lymphoproliferative or autoimmune disease. Conventionally attenuated androgens and antifibrinolytics have been used for prophylaxis, both for the long term and presurgically. Fresh frozen plasma and plasma-derived C1 inhibitor concentrate have been used primarily for treatment of acute attacks. All have drawbacks in side effects or potential for infection transmission. New treatments (recombinant C1 inhibitor, icatibant, DX-88, and for acquired angioedema, rituximab) so far show good safety profiles. Early data suggest these may be effective treatment alternatives. The efficacy of current treatment and the potential held by newer agents that target specific elements in complement or kinin pathways are examined. Some agents are likely to have a wider role in treatment of other, more common, forms of angioedema.
遗传性血管性水肿是一种常染色体显性疾病，临床上表现为反复发作的肿胀。它是由C1抑制剂产生不足或功能缺陷所致。获得性血管性水肿与淋巴增殖性疾病或自身免疫性疾病相关。传统上，减毒雄激素和抗纤溶药物已被用于长期预防和术前预防。新鲜冷冻血浆和血浆源性C1抑制剂浓缩物主要用于治疗急性发作。所有这些治疗方法都有副作用或感染传播风险等缺点。新的治疗方法（重组C1抑制剂、依库珠单抗、DX-88，以及用于获得性血管性水肿的利妥昔单抗）目前显示出良好的安全性。早期数据表明这些可能是有效的治疗替代方案。本文探讨了当前治疗方法的疗效以及针对补体或激肽途径中特定成分的新型药物的潜力。一些药物可能在治疗其他更常见的血管性水肿形式中发挥更广泛的作用。
