Zuraw B L, Sugimoto S, Curd J G
J Allergy Clin Immunol. 1986 Dec;78(6):1115-20. doi: 10.1016/0091-6749(86)90259-9.
The correct diagnosis and characterization of C1-inhibitor deficiency depends on both clinical observations and laboratory evaluation of complement in plasma. Rocket immunoelectrophoresis for C4d is a sensitive assay for C4 activation in plasma. We have evaluated the value of this assay in identifying patients with C1-inhibitor deficiency. C4 activation was assessed in the plasmas of 15 patients with hereditary angioedema, five patients with variant form of hereditary angioedema, and four patients with acquired C1-inhibitor deficiency. Control groups consisted of 27 patients with chronic idiopathic urticaria and/or angioedema and seven normal volunteers. C4 activation was detected in all 52 plasma samples collected from the 24 patients with C1-inhibitor deficiency. The degree of C4 activation increased during attacks of angioedema and decreased (but remained elevated) during treatment with attenuated androgens. The concentrations of C4, C2, and C1 inhibitor were also measured; however, none of these measurements identified all of the patients with C1-inhibitor deficiency. Thus, we conclude that the measurement of C4 activation is one of the best tests available to evaluate a patient for C1-inhibitor deficiency, and a normal result will exclude the diagnosis of C1-inhibitor deficiency.
C1抑制物缺乏症的正确诊断和特征描述取决于临床观察以及血浆补体的实验室评估。C4d的火箭免疫电泳是检测血浆中C4激活的一种灵敏方法。我们评估了该检测方法在识别C1抑制物缺乏症患者中的价值。对15例遗传性血管性水肿患者、5例遗传性血管性水肿变异型患者以及4例获得性C1抑制物缺乏症患者的血浆进行了C4激活评估。对照组包括27例慢性特发性荨麻疹和/或血管性水肿患者以及7名正常志愿者。在从24例C1抑制物缺乏症患者采集的所有52份血浆样本中均检测到了C4激活。在血管性水肿发作期间C4激活程度增加，而在用减毒雄激素治疗期间C4激活程度降低（但仍保持升高）。还测量了C4、C2和C1抑制物的浓度；然而，这些测量均未识别出所有C1抑制物缺乏症患者。因此，我们得出结论，C4激活检测是评估患者是否存在C1抑制物缺乏症的最佳检测方法之一，检测结果正常可排除C1抑制物缺乏症的诊断。
