Dipartimento di Scienze Cliniche L. Sacco, Università di Milano, Ospedale Luigi Sacco, Milano, Italy.
Curr Mol Med. 2010 Jun;10(4):354-60. doi: 10.2174/156652410791317066.
Acquired deficiency of C1 inhibitor (C1-INH) with angioedema symptoms (acquired angioedema, AAE) is characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. The mediator of symptoms is bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein a serine protease controlled by C1-INH. Autoantibodies inactivating C1-INH are detected in the majority of patients and account for the deficiency. Irrespectively to the presence of anti-C1-INH autoantibodies lymphoproliferative diseases, ranging from benign monoclonal gammopathies to malignant lymphoma, are frequently associated with AAE. Demonstration that monoclonal components correspond to anti-C1-INH autoantibodies and correlation between course of lymphoma and course of AAE provide strong support to consider the two diseases expression of the same pathologic process.
获得性 C1 抑制剂（C1-INH）缺乏伴血管性水肿症状（获得性血管性水肿，AAE）的特征是皮肤和胃肠道及口咽-喉黏膜局部血管通透性增加（血管性水肿）。症状的介质是缓激肽，一种强效血管活性肽，当它从高分子量激肽原被血浆激肽释放酶切割时释放出来，血浆激肽释放酶是一种受 C1-INH 控制的丝氨酸蛋白酶。大多数患者检测到失活 C1-INH 的自身抗体，这解释了缺乏的原因。无论是否存在抗 C1-INH 自身抗体，从良性单克隆丙种球蛋白病到恶性淋巴瘤的淋巴增生性疾病都常与 AAE 相关。证明单克隆成分与抗 C1-INH 自身抗体相对应，以及淋巴瘤病程与 AAE 病程之间的相关性，为将这两种疾病视为同一病理过程的表现提供了有力支持。
