Raj Naveen, Waters Barry
Department of Rheumatology, Nova Southeastern University/Larkin Hospital, Coral Springs, FL, USA.
Case Rep Rheumatol. 2016;2016:8219317. doi: 10.1155/2016/8219317. Epub 2016 Dec 15.
Activation of the classical pathway complement system has long been implicated in stimulating immune complex mediated tissue destruction in systemic lupus erythematosus (SLE). C3 and C4 complement levels are utilized as part of SLE diagnosis and monitoring criteria. Recently, cell bound complement activation products (CBCAPs) have shown increased sensitivity in diagnosing and monitoring lupus activity, compared to traditional markers. CBCAPs are increasingly utilized in rheumatology practice as additional serological markers in evaluating SLE patients. We report a case of a patient diagnosed with SLE that had chronically low C3 and C4, along with negative CBCAPs. We surmise that the patient has an inherited complement deficiency as the etiology of her SLE and that CBCAPs could be used to predict such deficiency.
长期以来,经典途径补体系统的激活一直被认为与系统性红斑狼疮(SLE)中免疫复合物介导的组织破坏有关。C3和C4补体水平被用作SLE诊断和监测标准的一部分。最近,与传统标志物相比,细胞结合补体激活产物(CBCAPs)在诊断和监测狼疮活动方面表现出更高的敏感性。CBCAPs在风湿病实践中越来越多地被用作评估SLE患者的额外血清学标志物。我们报告了一例被诊断为SLE的患者,其C3和C4长期偏低,且CBCAPs呈阴性。我们推测该患者存在遗传性补体缺陷,这是其SLE的病因,并且CBCAPs可用于预测此类缺陷。