Frémeaux-Bacchi V, Weiss L, Brun P, Kazatchkine M D
Service d'Immunologie, Hôpital Broussais, Paris, France.
Nephrol Dial Transplant. 1994;9(7):811-4.
The serum of patients with membranoproliferative glomerulonephritis (MPGN) often exhibits C3 nephritic factor activity (C3NeF) associated with autoantibodies directed against the C3bBb alternative complement pathway C3 convertase. In the present study, we have sequentially assessed C3NeF activity in the purified IgG fraction of the serum of a patient with MPGN type II and end-stage renal disease every month for 1 year following renal transplantation and bilateral nephrectomy. C3NeF activity in the patient's purified serum IgG decreased with time from the first month after transplantation and became undetectable after forty-five weeks. At that time, plasma levels of C3 and factor B had returned to normal values. The disappearance of C3NeF activity in patient's IgG was selective in that no change in activity of two other autoantibodies and of one antibody against a non-self antigen was observed with time, indicating that the decrease in C3NeF activity was not related to a non-specific effect of immunosuppressive therapy. Loss of C3NeF activity was not related to the generation of anti-idiotypic antibodies against C3NeF. This study documents for the first time the selective disappearance of C3NeF activity following renal transplantation and bilateral nephrectomy. The results suggest that the autoantibody represents an antigen-driven expansion of self reactive B cell clones in response to a specific disease process in the kidney of patients with MPGN.
膜增生性肾小球肾炎(MPGN)患者的血清常表现出与针对C3bBb替代补体途径C3转化酶的自身抗体相关的C3肾炎因子活性(C3NeF)。在本研究中,我们对一名患有II型MPGN和终末期肾病的患者在肾移植和双侧肾切除术后1年每月依次评估其血清纯化IgG组分中的C3NeF活性。患者纯化血清IgG中的C3NeF活性自移植后第一个月起随时间下降,在45周后变得无法检测到。此时,C3和B因子的血浆水平已恢复到正常数值。患者IgG中C3NeF活性的消失具有选择性,因为未观察到其他两种自身抗体以及一种针对非自身抗原的抗体的活性随时间发生变化,这表明C3NeF活性的降低与免疫抑制治疗的非特异性效应无关。C3NeF活性的丧失与针对C3NeF的抗独特型抗体的产生无关。本研究首次记录了肾移植和双侧肾切除术后C3NeF活性的选择性消失。结果表明,自身抗体代表了MPGN患者肾脏中特定疾病过程引发的自身反应性B细胞克隆的抗原驱动性扩增。