Zuraw B L, Altman L C
Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, CA 92037.
J Allergy Clin Immunol. 1991 Dec;88(6):908-18. doi: 10.1016/0091-6749(91)90248-m.
Acquired C1-inhibitor (C1 INH) deficiency is usually found in association with an underlying disease that is believed to be responsible for increased C1 INH catabolism, ultimately leading to the development of C1 INH deficiency. We report a remarkable patient with acquired C1 INH deficiency in whom a unique progression of complement- and contact-system abnormalities has been observed. S. G. suffers from recurrent episodes of angioedema and hypotension. Results of repeated complement studies were initially normal, and the patient was diagnosed as having idiopathic anaphylaxis. Two years later, the patient was found to develop acute consumption of C1 INH with activation of the complement and contact systems during episodes of angioedema. The patient continued to have normal C1 INH levels and to have no evidence for complement- or contact-system activation between attacks of angioedema. One year later, her course evolved into a more typical course for acquired C1 INH deficiency consisting of continuously low functional C1 INH levels with evidence of activation of the complement and contact systems. S. G. provides a unique insight into the development of acquired C1 INH deficiency.
获得性C1抑制物（C1 INH）缺乏症通常与一种潜在疾病相关，该疾病被认为是导致C1 INH分解代谢增加的原因，最终导致C1 INH缺乏症的发生。我们报告了一位患有获得性C1 INH缺乏症的特殊患者，在该患者中观察到了补体和接触系统异常的独特进展。S.G.患有复发性血管性水肿和低血压。重复进行的补体研究结果最初正常，该患者被诊断为特发性过敏反应。两年后，发现该患者在血管性水肿发作期间出现C1 INH急性消耗，并伴有补体和接触系统激活。该患者在血管性水肿发作之间的C1 INH水平持续正常，且没有补体或接触系统激活的证据。一年后，她的病程演变为获得性C1 INH缺乏症更典型的病程，即功能性C1 INH水平持续降低，并伴有补体和接触系统激活的证据。S.G.为获得性C1 INH缺乏症的发展提供了独特的见解。
