Department of Nephrology, Abdominal Center, Helsinki University Central Hospital, Helsinki, Finland.
Department of Nephrology, Abdominal Center, Helsinki University Central Hospital, Helsinki, Finland.
Clin Immunol. 2018 Feb;187:104-106. doi: 10.1016/j.clim.2017.10.013. Epub 2017 Oct 31.
There is a great lack of efficient treatments for membranoproliferative glomerulonephritis (MPGN) and recently emerged complement therapies have been proposed to be useful. We report a patient with a complement-mediated MPGN having recurrencies in kidney allografts and an unsuccessful treatment with complement inhibitor, eculizumab (anti-C5 monoclonal antibody). Nephritic factor (C3Nef), an autoantibody against C3bBb, in the patient serum activated C3 but not C5 showing that major damage was mediated by C3 activation with clearly less involvement of C5 explaining unresponsiveness to eculizumab. Analyzing C3Nef-mediated C3 and C5 activation separately could help in choosing the right patients for eculizumab therapy.
目前针对膜增生性肾小球肾炎（MPGN）的有效治疗方法非常有限，最近出现的一些补体治疗方法被认为可能有效。我们报告了一例补体介导的 MPGN 患者，其肾移植后复发，且补体抑制剂依库珠单抗（抗 C5 单克隆抗体）治疗无效。患者血清中的肾炎因子（C3Nef）是一种针对 C3bBb 的自身抗体，可激活 C3 但不激活 C5，表明主要的损伤是由 C3 激活介导的，C5 的参与明显较少，这解释了依库珠单抗治疗无效的原因。分别分析 C3Nef 介导的 C3 和 C5 激活可能有助于为依库珠单抗治疗选择合适的患者。
