Angioedema due to acquired C1-inhibitor deficiency: A bridging condition between autoimmunity and lymphoproliferation
Angioedema due to acquired C1-inhibitor deficiency: A bridging condition between autoimmunity and lymphoproliferation
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DOI：
10.1016/j.autrev.2008.05.003
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发表时间：
2008-12-01
影响因子：
13.6
通讯作者：
Cicardi, Marco
中科院分区：
文献类型：
--
作者：
Cugno, Massimo;Castelli, Roberto;Cicardi, Marco
由于人补体第一成分抑制剂（CI-INH）的获得性缺乏引起的血管性水肿是一种罕见的综合征，通常被鉴定为获得性血管性水肿（AAE）。CI-INH缺乏症的临床特征也可能是遗传性的（遗传性血管性水肿，HAE），包括皮下非炎性肿胀、上呼吸道受累和由于胃肠道部分梗阻引起的腹痛。与HAE患者不同，AAE患者无血管性水肿家族史，其特征为症状迟发和由于C1-INH的高钾化引起的对治疗的各种反应。C1-INH功能的降低导致经典补体途径的激活和补体消耗，以及接触系统的激活，导致血管活性肽缓激肽的产生、血管通透性增加和血管性水肿。AAE通常与淋巴组织增生性疾病相关，从意义不明的单克隆丙种球蛋白病（MGUS）到非霍奇金淋巴瘤（NHL）和/或抗C1-INH灭活自身抗体。真正的B细胞恶性肿瘤、非恶性B细胞增殖和致病性自身免疫反应的共存表明，AAE患者都受到改变的B细胞增殖控制的影响，尽管他们的临床演变可能不同。(C)2008 Elsevier B. V.保留所有权利。
Angioedema due to an acquired deficiency in the inhibitor of the first component of human complement (CI-INH) is a rare syndrome that is usually identified as acquired angioedema (AAE). The clinical features of CI-INH deficiency, which may also be of genetic origin (hereditary angioedema, HAE), include subcutaneous, non-pruritic swelling, involvement of the upper respiratory tract, and abdominal pain due to partial obstruction of the gastrointestinal tract. Unlike those with HAE, AAE patients have no family history of angioedema and are characterised by the late onset of symptoms and various responses to treatment due to the hypercatabolism of C1-INH. The reduction in C1-INH function leads to activation of the classical complement pathway and complement consumption, as well as activation of the contact system leading to the generation of the vasoactive peptide bradykinin, increased vascular permeability, and angioedema. AAE is frequently associated with lymphoproliferative diseases ranging from monoclonal gammopathies of uncertain significance (MGUS) to non-Hodgkin's lymphoma (NHL) and/or anti-C1-INH inactivating autoantibodies. The coexistence of true B cell malignancy, non-malignant B cell proliferation and pathogenic autoimmune responses suggests that AAE patients are all affected by altered B cell proliferation control although their clinical evolution may vary. (C) 2008 Elsevier B.V. All rights reserved.