Abstract:
This study elucidated the etiology of C3 glomerulonephritis (C3GN) and non-C3GN with primary membranoproliferative glomerulonephritis (MPGN) using transmission electron microscopy (TEM) and periodic acid-methenamine silver stain (PAM-EM). Thirty-one primary MPGN cases were analyzed by TEM and PAM-EM to distinguish among MPGN I, MPGN II, MPGN III Burkholder subtype (MPGN IIIB), and Anders and Strife subtype (MPGN IIIA/S). Each case was also classified into C3GN or non-C3GN according to the standard C3GN definition using immunostaining. Four cases of MPGN II met C3 glomerulopathy; whereas, four cases of MPGN IIIB did not meet C3 glomerulopathy. Seven of 11 cases (64%) of MPGN I without GBM disruption and 7 of 12 cases (58%) of MPGN IIIA/S with GBM disruption met the non-C3GN criteria with significant immunoglobulins\' deposition. Regardless of the C3GN or non-C3GN diagnosis, the deposits in primary MPGN I and MPGN IIIA/S exhibited ill-defined, amorphous, and foggy characteristics similar to those found in postinfectious GN but were different from immune complex (IC) deposits seen in MPGN IIIB. Not only C3GN but also non-C3GN was due to mechanisms other than IC deposition as found in postinfectious GN. Consequently, GBM disruption of MPGN IIIA/S was not due to IC deposition.
摘要:
本研究使用透射电子显微镜(TEM)和高碘酸-甲胺银染(PAM-EM)阐明了C3肾小球肾炎(C3GN)和非C3GN合并原发性膜增生性肾小球肾炎(MPGN)的病因。通过TEM和PAM-EM分析了31例原发性MPGN病例,以区分MPGNI。MPGNII,MPGNIIIBurkholder亚型(MPGNIIIB),以及Anders和Strife亚型(MPGNIIIA/S)。根据使用免疫染色的标准C3GN定义,每个病例还被分类为C3GN或非C3GN。4例MPGNII符合C3肾小球病;然而,4例MPGNIIIB患者未符合C3肾小球疾病。无GBM破坏的MPGNI11例中有7例(64%),GBM破坏的MPGNIIIA/S12例中有7例(58%)符合非C3GN标准,具有显着的免疫球蛋白沉积。无论C3GN还是非C3GN诊断,初级MPGNI和MPGNIIIA/S中的矿床表现出不明确的特征,无定形,和雾状特征与感染后的GN相似,但与MPGNIIIB中的免疫复合物(IC)沉积物不同。不仅C3GN,而且非C3GN也是由于感染后GN中发现的IC沉积以外的机制。因此,MPGNIIIA/S的GBM破坏不是由于IC沉积。