Nishida M, Kawakatsu H, Komatsu H, Ishiwari K, Tamai M, Sawada T
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Am J Kidney Dis. 2000 May;35(5):E22. doi: 10.1016/s0272-6386(00)70287-3.
A 17-year-old girl showed mild proteinuria accompanied by hematuria and mild hypocomplementemia. A light microscopic study of the first renal biopsy specimen showed diffuse mild to moderate mesangial proliferation and thickening of the glomerular basement membrane (GBM). An immunofluorescence study showed dominant positive staining (3+) of IgG and C1q in the glomerular mesangium and capillary loop. Staining for C3 and fibrinogen was weak or 1+. Staining for IgA and IgM was negative. Electron-dense deposits were present in the mesangial area and also in the subepithelial, subendothelial, and intramembranous space. Urinary findings improved after dipyridamole treatment. The second renal biopsy, which was performed 5 years later, showed histological improvements, and various pictures of washing-out of deposits were also noted in an electron microscopic study. However, dominant positive staining for IgG and C1q was persistent in an immunofluorescence study. The glomerulopathy of this case belongs in the criteria of neither membranoproliferative glomerulonephritis nor lupus nephritis but could be designated as C1q nephropathy. This is the first report of a histological improvement in C1q nephropathy.
一名17岁女孩出现轻度蛋白尿,伴有血尿和轻度补体降低。对首次肾活检标本进行的光镜检查显示弥漫性轻度至中度系膜增生和肾小球基底膜(GBM)增厚。免疫荧光研究显示肾小球系膜和毛细血管袢中IgG和C1q呈显性阳性染色(3+)。C3和纤维蛋白原染色较弱或为1+。IgA和IgM染色为阴性。电子致密沉积物存在于系膜区以及上皮下、内皮和膜内间隙。双嘧达莫治疗后尿液检查结果有所改善。5年后进行的第二次肾活检显示组织学改善,电镜研究中也观察到沉积物洗脱的各种表现。然而,免疫荧光研究中IgG和C1q的显性阳性染色持续存在。该病例的肾小球病既不符合膜增生性肾小球肾炎的标准,也不符合狼疮性肾炎的标准,但可诊断为C1q肾病。这是C1q肾病组织学改善的首例报告。