Markowitz G S, Kambham N, Maruyama S, Appel G B, Cohen D J, Kim R C, Andres G A, D'Agati V D
Department of Pathology, Columbia Presbyterian Medical Center, New York, NY, USA.
Clin Nephrol. 2000 Dec;54(6):478-86.
Bowman's capsular and tubular basement membrane (TBM) deposits are an extremely unusual finding in non-lupus membranous glomerulopathy (MGN). We report three atypical cases of MGN with abundant Bowman's capsular and TBM deposits. In two cases, MGN was idiopathic; in the third case, MGN occurred in the renal allograft in the setting of HCV seropositivity. In addition to the usual glomerular capillary wall deposits, immunofluorescence and electron microscopy revealed extensive immune deposits within Bowman's capsule and TBMs, predominantly at the base of parietal and tubular epithelial cells. These cases suggest a potential pathomechanism of autoantibody to secreted epithelial antigens shared by visceral, parietal, and tubular epithelial cells. In all three cases, indirect immunofluorescence was unable to detect autoantibody to normal renal epithelial or matrix constituents. Furthermore, ELISA was unable to demonstrate circulating antibody to major extracellular matrix components. The implications of these findings for the pathogenesis of MGN are explored.
鲍曼囊和肾小管基底膜(TBM)沉积物在非狼疮性膜性肾小球病(MGN)中是极为罕见的发现。我们报告了3例伴有大量鲍曼囊和TBM沉积物的非典型MGN病例。其中2例MGN为特发性;第3例MGN发生于丙型肝炎病毒血清学阳性的肾移植受者。除了常见的肾小球毛细血管壁沉积物外,免疫荧光和电子显微镜检查显示鲍曼囊和TBM内有广泛的免疫沉积物,主要位于壁层和肾小管上皮细胞底部。这些病例提示了针对内脏、壁层和肾小管上皮细胞共有的分泌性上皮抗原的自身抗体的潜在发病机制。在所有3例病例中,间接免疫荧光未能检测到针对正常肾上皮或基质成分的自身抗体。此外,酶联免疫吸附测定未能证实存在针对主要细胞外基质成分的循环抗体。本文探讨了这些发现对MGN发病机制的意义。