Komatsuda Atsushi, Wakui Hideki, Ohtani Hiroshi, Nimura Takashi, Sawada Ken-Ichi
Department of Hematology, Nephrology, and Rheumatology , Akita University Graduate School of Medicine , Akita , Japan.
Department of Nephrology and Dialysis , Akita Kumiai General Hospital , Japan.
NDT Plus. 2010 Aug;3(4):357-9. doi: 10.1093/ndtplus/sfq076. Epub 2010 May 2.
A 78-year-old woman developed acute-onset nephrotic syndrome. A renal biopsy showed mild mesangial proliferative glomerulonephritis. Immunofluorescence studies revealed granular IgG3- λ deposits within the mesangial area and along the glomerular capillary walls. Electron microscopy showed mesangial and subendothelial granular electron-dense deposits. The pattern of deposition was predominantly mesangial. Serum or urine monoclonal proteins were not detected. Middle-dose steroid therapy induced a rapid remission of nephrotic syndrome. We consider that this is the first case of steroid-responsive nephrotic syndrome due to an extremely rare glomerular disease, proliferative glomerulonephritis with monoclonal IgG deposits associated with pure mesangial proliferative features.
一名78岁女性突发急性肾病综合征。肾活检显示为轻度系膜增生性肾小球肾炎。免疫荧光研究显示系膜区及沿肾小球毛细血管壁有颗粒状IgG3-λ沉积。电子显微镜检查显示系膜和内皮下有颗粒状电子致密沉积物。沉积模式主要为系膜型。未检测到血清或尿单克隆蛋白。中剂量类固醇治疗使肾病综合征迅速缓解。我们认为这是首例因一种极其罕见的肾小球疾病——具有纯系膜增生特征的伴单克隆IgG沉积的增生性肾小球肾炎——导致的类固醇反应性肾病综合征病例。
