Hashimoto Seiji, Ogawa Yayoi, Ishida Takashi, Mochizuki Toshio, Koike Takao, Sato Hidetoshi, Ueda Takahiro
Department of Nephrology Division, Sapporo General City Hospital, Sapporo, Japan.
Clin Exp Nephrol. 2004 Sep;8(3):266-9. doi: 10.1007/s10157-004-0282-2.
A 21-year-old woman showed heavy proteinuria and edema. A light microscopic study of a renal biopsy specimen showed diffuse mild mesangial expansion, with borderline mesangial hypercellularity. An immunofluorescence study revealed dominant positive staining (3+) of C1q in the glomerular mesangium. Stainings for C3, C4, IgG, and IgM were weak or 1+. Staining for IgA was negative. Electron-dense deposits were present in the mesangial area. There was significant fusion of foot processes. There was no serological or clinical evidence of collagen disease. She was treated with oral prednisolone (initially, 40 mg/day). The proteinuria was alleviated and the patient remains in complete remission. The histopathological studies were compatible with C1q nephropathy, although the clinical outcome differed in a number of aspects. The clinical picture in the current patient appears to represent a very rare phenotype of nephritis.
一名21岁女性出现大量蛋白尿和水肿。对肾活检标本进行的光镜检查显示弥漫性轻度系膜扩张，系膜细胞增生处于临界状态。免疫荧光研究显示肾小球系膜中C1q呈显性阳性染色（3+）。C3、C4、IgG和IgM染色较弱或为1+。IgA染色为阴性。系膜区存在电子致密沉积物。足突有明显融合。没有胶原病的血清学或临床证据。她接受了口服泼尼松龙治疗（初始剂量为40mg/天）。蛋白尿得到缓解，患者仍处于完全缓解状态。组织病理学研究与C1q肾病相符，尽管临床结果在多个方面有所不同。当前患者的临床表现似乎代表了一种非常罕见的肾炎表型。
