Yamamoto Tatsuo, Togawa Akashi, Eguchi Masanobu, Ohashi Naro, Yasuda Hideo, Harita Yutaka, Hattori Motoshi, Yamaguchi Yutaka, Ohyama Kunio
Division of Nephrology, Fujieda Municipal General Hospital, 4-1-11 Surugadai, Fujieda, Shizuoka, 426-8677, Japan.
Division of Nephrology, Shizuoka Saiseikai General Hospital, Shizuoka, Japan.
CEN Case Rep. 2016 Nov;5(2):163-167. doi: 10.1007/s13730-016-0217-2. Epub 2016 Mar 8.
A 62-year-old woman with nephrotic syndrome underwent a renal biopsy. Under light microscopy, the biopsy findings included lobulation and enlargement of glomeruli, occasional thickening of glomerular capillary walls, and narrowing of the capillary lumen by swollen endothelial cells. Congo red staining was negative for amyloid. No significant intraglomerular fibrin deposition was found by phosphotungstic acid hematoxylin staining. Immunofluorescence microscopy showed no deposition of immunoglobulin G, A, or M; no κ or λ light chains; and no C3 or C1q. Electron microscopy revealed distinctive subendothelial and mesangial fibrillar deposits, mesangial cell interposition, and swelling and vacuolization of endothelial cells resulting in capillary lumen narrowing. Although some curvilinear fibrillar deposits mimicked the bundles of type III collagen fibers seen in collagenofibrotic glomerulopathy, neither glomerular deposition of type III collagen nor elevation of serum procollagen III peptide was noted. This glomerulopathy does not fulfill any known disease entities with non-amyloid non-immunoglobulin-derived organized glomerular deposits.
一名患有肾病综合征的62岁女性接受了肾活检。在光学显微镜下，活检结果包括肾小球分叶和增大、肾小球毛细血管壁偶尔增厚，以及肿胀的内皮细胞导致毛细血管腔狭窄。刚果红染色显示淀粉样蛋白阴性。磷钨酸苏木精染色未发现明显的肾小球内纤维蛋白沉积。免疫荧光显微镜检查未显示免疫球蛋白G、A或M沉积；未发现κ或λ轻链；也未发现C3或C1q。电子显微镜显示有独特的内皮下和系膜纤维状沉积物、系膜细胞插入，以及内皮细胞肿胀和空泡化导致毛细血管腔狭窄。尽管一些曲线状纤维状沉积物类似于胶原纤维性肾小球病中所见的III型胶原纤维束，但未发现III型胶原在肾小球沉积，血清前胶原III肽也未升高。这种肾小球病不符合任何已知的具有非淀粉样非免疫球蛋白来源的有组织的肾小球沉积物的疾病实体。
