Akai Y, Shiiki H, Morimoto E, Iwano M, Fujii Y, Dohi K, Ishikawa H
First Department of Internal Medicine, Nara Medical University.
Nihon Jinzo Gakkai Shi. 1992 Aug;34(8):939-44.
A male aged 58 was admitted to our hospital because of proteinuria, hematuria and bilateral pretibial edema. Laboratory tests showed normocytic, normochromic anemia and moderately impaired renal function. Antinuclear antibodies were negative. Neither M-protein nor Bence-Jones protein were detected. Light microscopic study on the biopsied renal specimen indicated a moderate mesangial proliferation accompanying with the deposition of PAS-positive and Congo red-negative materials in the subendothelial area. C3 accumulated segmentally along the capillary walls, which was clarified by immunofluorescence microscopy. Staining for IgG, IgA, IgM and light chains were negative. Electron microscopy demonstrated the deposition of microtubules in the mesangial, subepithelial and subendothelial areas. The diameter of these microtubules ranged from 40 to 80 nm. Such type of the microtubules have been reported to exist in the glomeruli in the patients with systemic diseases such as amyloidosis, systemic lupus erythematosus, cryoglobulinemia and light chain disease. In our patient, however, any clinical or serological findings suggestive of these systemic diseases were not obtained. On the other hand previous report pointed out that microtubules deposited in the glomeruli in the patients with immunotactoid glomerulopathy or other glomerulopathies. Our patient had the clinical features consistent with these glomerulopathies. However, no depositions of immunoglobulins were observed. This case is an atypical glomerulopathy accompanying with the glomerular microtubular deposits.
一名58岁男性因蛋白尿、血尿和双侧胫前水肿入院。实验室检查显示正细胞正色素性贫血和肾功能中度受损。抗核抗体阴性。未检测到M蛋白和本周氏蛋白。对肾活检标本的光镜研究显示中度系膜增生，伴有内皮下区域PAS阳性和刚果红阴性物质沉积。免疫荧光显微镜检查显示C3沿毛细血管壁节段性积聚。IgG、IgA、IgM和轻链染色均为阴性。电子显微镜显示微管沉积于系膜、上皮下和内皮下区域。这些微管直径为40至80纳米。据报道，在淀粉样变性、系统性红斑狼疮、冷球蛋白血症和轻链病等系统性疾病患者的肾小球中存在这种类型的微管。然而，在我们的患者中，未获得任何提示这些系统性疾病的临床或血清学发现。另一方面，先前的报告指出，在免疫触须样肾小球病或其他肾小球病患者的肾小球中存在微管沉积。我们的患者具有与这些肾小球病一致的临床特征。然而，未观察到免疫球蛋白沉积。该病例是一种伴有肾小球微管沉积的非典型肾小球病。
