Farkas H, Gyeney L, Majthényi P, Füst G, Varga L
Department of Otorhinolaryngology, National Institute of Rheumatology and Physiotherapy, Budapest, Hungary.
Z Gastroenterol. 1999 Jun;37(6):513-8.
The first component of the classical pathway of the complement system, C1 is regulated by a serum protein, the C1-esterase inhibitor (C1-INH). Deficiency of this protein leads to the release of vasoactive mediators (C2 kinin and bradykinin) that increase vascular permeability and can induce edema formation in subcutaneous and submucosal tissues. The genetic variant of C1-INH deficiency is inherited as an autosomal dominant trait and causes hereditary angioneurotic edema. The acquired form of C1-INH deficiency is characterized by similar manifestations and can occur in association with lymphoproliferative diseases, malignancy, immune disorders, and infections. The authors present a case of acquired C1-INH deficiency in a patient with Helicobacter pylori infection. Complete eradication of this pathogen was followed by the resolution of symptoms and normalization of serum complement levels. It seems therefore probable that in this patient, acquired C1-INH deficiency was related to Helicobacter pylori infection. To our best knowledge, no similar observations have been published so far. Specific immune reactions are important contributing factors in H. pylori. Excessive consumption of complement by antibodies directed against H. pylori is a potential cause of C1-INH deficiency observed in our case.
补体系统经典途径的首个成分C1受一种血清蛋白C1酯酶抑制剂（C1-INH）调控。该蛋白缺乏会导致血管活性介质（C2激肽和缓激肽）释放，从而增加血管通透性，并可在皮下和黏膜下组织诱发水肿形成。C1-INH缺乏的遗传变异以常染色体显性性状遗传，可导致遗传性血管性水肿。C1-INH缺乏的后天形式具有相似表现，可与淋巴增殖性疾病、恶性肿瘤、免疫紊乱及感染相关。作者报告了1例幽门螺杆菌感染患者后天性C1-INH缺乏的病例。该病原体被彻底根除后，症状得以缓解，血清补体水平恢复正常。因此，该患者后天性C1-INH缺乏可能与幽门螺杆菌感染有关。据我们所知，目前尚未发表过类似观察结果。特异性免疫反应是幽门螺杆菌感染的重要促成因素。针对幽门螺杆菌的抗体过度消耗补体是我们病例中观察到的C1-INH缺乏的一个潜在原因。
