Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.
Kidney Int. 2013 Jan;83(1):129-37. doi: 10.1038/ki.2012.313. Epub 2012 Aug 22.
Studies in animal models suggest that complement activation is crucial in the pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Here we investigate the circulating complement activation profile of 66 patients with active stage AAV compared to that of 54 patients with AAV in remission. Plasma levels of C3a, C5a, soluble C5b-9, and Bb, all determined by enzyme-linked immunosorbent assay, were significantly higher in active stage than in remission of AAV, while plasma levels of properdin were significantly lower in the former than the latter disease stage. There was no significant difference in the plasma levels of C4d between active stage and remission. The plasma level of Bb in patients with active AAV significantly correlated with the proportion of total and cellular crescents in the renal biopsy, the erythrocyte sedimentation rate, and the Birmingham Vasculitis Activity Scores. Thus, systemic activation of complement by the alternative pathway takes place in human AAV. Circulating Bb might be a useful biomarker in assessing disease activity of AAV.
研究动物模型表明，补体激活在抗中性粒细胞胞浆抗体相关性血管炎（AAV）的发病机制中至关重要。在这里，我们研究了 66 例活动期 AAV 患者与 54 例缓解期 AAV 患者的循环补体激活谱。通过酶联免疫吸附试验测定的 C3a、C5a、可溶性 C5b-9 和 Bb 的血浆水平在活动期 AAV 中明显高于缓解期，而前一疾病阶段的血浆水平 Properdin 明显低于后者。活动期与缓解期之间 C4d 的血浆水平无显著差异。活动期 AAV 患者的 Bb 血浆水平与肾活检中总新月体和细胞新月体的比例、红细胞沉降率和伯明翰血管炎活动评分显著相关。因此，旁路途径的系统性补体激活发生在人类 AAV 中。循环 Bb 可能是评估 AAV 疾病活动的有用生物标志物。
