Ohi H, Yasugi T
Department of Internal Medicine II, Nihon University School of Medicine, Tokyo, Japan.
Clin Exp Immunol. 1994 Feb;95(2):316-21. doi: 10.1111/j.1365-2249.1994.tb06530.x.
One hundred patients diagnosed with hypocomplementaemic MPGN (C3 < 40%) were studied to determine the presence of C3 nephritic factor (C3NeF) and/or C4 nephritic factor (C4NeF). Of those studied, 12 were C3NeF-positive, nine were C4NeF-positive and 10 were positive for both C3NeF and C4NeF. In the 10 patients both C3NeF- and C4NeF-positive, a marked decrease in C3 and C5 levels and a decrease in levels of late components from C6 to C9 were observed. This observation was in contrast to that seen in patients who were either C3NeF- or C4NeF-positive. Patients positive for both C3NeF and C4NeF continued to exhibit hypocomplementaemia after therapy. Immunofluorescent findings revealed heavy C3 immunoglobulin deposits in the 10 patients who were both C3NeF- and C4NeF-positive, whereas no such deposits were found in those patients who were either C3NeF- or C4NeF-positive only. When those patients who were both C3NeF- and C4NeF-positive were compared with those who were either C3NeF- or C4NeF-positive, nephritic syndrome and a poor prognosis were observed more frequently. This study demonstrates a correlation between clinical outcome and hypocomplementaemic MPGN. Further investigations of MPGN as an autoimmune disease are necessary.
对100例诊断为低补体血症性膜增生性肾小球肾炎(C3<40%)的患者进行研究,以确定是否存在C3肾炎因子(C3NeF)和/或C4肾炎因子(C4NeF)。在这些研究对象中,12例C3NeF阳性,9例C4NeF阳性,10例C3NeF和C4NeF均为阳性。在这10例C3NeF和C4NeF均为阳性的患者中,观察到C3和C5水平显著降低,以及C6至C9晚期补体成分水平下降。这一观察结果与C3NeF或C4NeF阳性患者的情况形成对比。C3NeF和C4NeF均为阳性的患者在治疗后仍表现为低补体血症。免疫荧光检查结果显示,在10例C3NeF和C4NeF均为阳性的患者中存在大量C3免疫球蛋白沉积,而在仅C3NeF或C4NeF阳性的患者中未发现此类沉积。当将C3NeF和C4NeF均为阳性的患者与仅C3NeF或C4NeF阳性的患者进行比较时,发现肾病综合征和预后不良更为常见。本研究表明临床结局与低补体血症性膜增生性肾小球肾炎之间存在相关性。有必要对膜增生性肾小球肾炎作为一种自身免疫性疾病进行进一步研究。