Kronz J D, Neu A M, Nadasdy T
Department of Pathology, The Johns Hopkins University, Baltimore, MD 21205, USA.
Clin Nephrol. 1998 Oct;50(4):218-23.
In addition to fibrillary glomerulonephritis (FGN), Congo red negative mesangial fibrils may commonly be seen in sclerosing glomerular diseases. Rarely, these nonspecific mesangial fibrils (NMF) may mimic fibrils in FGN and cause a differential diagnostic pitfall. Following an interesting case of sclerosing crescentic glomerulonephritis with abundant NMF (which is presented in some detail) we have reviewed our renal biopsy files for a period of two and a half years and found additional 16 cases where the presence of NMF warranted studies to exclude FGN and other diseases with fibrillary deposits. The immunofluorescence pattern characteristically seen in FGN was not present in any of these cases. Our data confirm that mesangial fibrillary material seen ultrastructurally in sclerosing glomeruli with negative or nonspecific immunofluorescence (IF) represents a nonspecific reaction of the mesangial matrix to chronic glomerular injury. The presence of NMF should not lead to the erroneous diagnosis of FGN. Negative or nonspecific immunofluoresence, localization to the mesangium in a usually segmental fashion, and the more bundle-like than random arrangement of fibrils are helpful diagnostic hints in differentiating NMF from fibrils in FGN.
除了纤维性肾小球肾炎（FGN）外，刚果红阴性的系膜纤维在硬化性肾小球疾病中也较为常见。这些非特异性系膜纤维（NMF）偶尔会与FGN中的纤维相似，导致诊断上的陷阱。在报告了一例伴有大量NMF的硬化性新月体性肾小球肾炎的有趣病例（将进行一些详细介绍）后，我们回顾了两年半期间的肾活检档案，发现另外16例存在NMF的病例，这些病例需要进行研究以排除FGN和其他伴有纤维样沉积物的疾病。在这些病例中均未出现FGN典型的免疫荧光模式。我们的数据证实，在具有阴性或非特异性免疫荧光（IF）的硬化性肾小球中，超微结构所见的系膜纤维物质代表系膜基质对慢性肾小球损伤的非特异性反应。NMF的存在不应导致FGN的错误诊断。阴性或非特异性免疫荧光、通常呈节段性定位于系膜以及纤维束状排列而非随机排列，这些都是有助于将NMF与FGN中的纤维区分开来的诊断线索。
