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不同C1q - CIC和C3浓度的系统性红斑狼疮(SLE)患者的临床管理

Clinical management of patients with systemic lupus erythematosus (SLE) with different C1q-CIC and C3 concentrations.

作者信息

Karamehic Jasenko, Subasic Djemo, Kasumovic Mersija, Hodzic Harun, Prljaca-Zecevici Lamija, Tufekcic Mersiha, Aganovic-Musinovic Izeta

机构信息

Institute of Clinical immunology-Clinical Centre, University of Sarajevo, Bolnicka 25, 71000 Sarajevo, Bosnia and Herzegovina.

出版信息

Med Arh. 2010;64(2):75-9.

Abstract

Over the third of SLE (Systemic Lupus Erythematosus) patients have a high level auto-antibodies-antigen complex that contains some complement proteins, especially C1q as the trigger protein in the classical complement activation pathway. So, the SLE, as an autoimmune disease, is certainly related to disorders caused by activation of complement system, that finally leads to tissue damage. It may also be caused by hereditary deficiency (complement genes mutations). In such case, some components of the complement system might be inactivated. There are mutations that cause disorders in each of three complement system activation pathways (classical, alternative and lectin).The serum samples of SLE patients show the presence of specific autoantibodies for some complement components. Today, for clinical management of SLE patients, determination of level of C1q-CIC and C3 complement component in serum specimens have great diagnostic and therapeutic importance. During the year 2000, we analyzed a numerous serum samples from patients suspected to autoimmune diseases (SLE especially). The samples were collected from several clinics in the Clinical Center of University of Sarajevo, mostly from Clinic of Infectious Diseases, Pediatrics, Internal Medicine and Gastroenterohepatology Clinic. Primary samples went through screening for the presence of ANA using ANA-IFA method and further characterization of ANA positive samples was carried out using IFA-ANA titration, ELISA and nephelometry.

摘要

超过三分之一的系统性红斑狼疮(SLE)患者体内存在高水平的自身抗体 - 抗原复合物,该复合物包含一些补体蛋白,尤其是作为经典补体激活途径触发蛋白的C1q。因此,作为一种自身免疫性疾病,SLE肯定与补体系统激活所导致的紊乱有关,最终会导致组织损伤。它也可能由遗传性缺陷(补体基因突变)引起。在这种情况下,补体系统的一些成分可能会失活。在补体系统的三种激活途径(经典途径、替代途径和凝集素途径)中,每种途径都存在导致紊乱的突变。SLE患者的血清样本显示存在针对某些补体成分的特异性自身抗体。如今,对于SLE患者的临床管理,测定血清标本中C1q - CIC和C3补体成分的水平具有重要的诊断和治疗意义。在2000年期间,我们分析了大量疑似自身免疫性疾病(尤其是SLE)患者的血清样本。这些样本取自萨拉热窝大学临床中心的几家诊所,大部分来自传染病诊所、儿科、内科和胃肠肝病诊所。首先使用ANA - IFA方法对样本进行抗核抗体(ANA)筛查,然后使用IFA - ANA滴定、酶联免疫吸附测定(ELISA)和比浊法对ANA阳性样本进行进一步鉴定。

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