Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.
Clin Exp Nephrol. 2012 Jun;16(3):473-9. doi: 10.1007/s10157-011-0580-4. Epub 2012 Jan 19.
A 50-year-old woman with a 1-month history of lower extremity edema and a 5 kg weight increase was admitted to our hospital with suspected nephrotic syndrome in October 1999. Urine protein level was 3.5 g per day, 10-15 erythrocytes in urine per high-power field, and serum albumin level 2.5 g/dl. Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray. The results of a renal biopsy indicated slight mesangial proliferation in the glomeruli by light microscopy, and an immunofluorescence study confirmed the deposition of immunoglobulin (Ig) A and C3 in the mesangial area. Diffuse attenuation of foot processes and dense deposits in the mesangial area were observed by electron microscopy. Treatment with 40 mg/day of prednisolone was effective, and proteinuria was negative 1 month later. Because of this course, we diagnosed minimal change nephrotic syndrome complicated by mild-proliferative IgA nephropathy. In November 2000, there was a relapse of nephrotic syndrome, which was believed to be induced by an influenza vaccination, but response to increased steroid treatment was favorable, and proteinuria disappeared on day 13 of steroid increase. A second relapse in May 2001, showed steroid resistance with renal insufficiency, and an increase in the selectivity index to 0.195. Light microscopy revealed focal sclerotic lesions of the glomeruli, and an immunofluorescence study revealed attenuation of mesangial IgA and C3 deposition. These findings led to the diagnosis that minimal change nephrotic syndrome had transitioned to focal segmental glomerulosclerosis, whereby mesangial IgA deposition was reduced by immunosuppressive treatment. Subsequently, her renal function gradually worsened to the point of end-stage renal failure by 27 months after the second relapse of nephrotic syndrome.
一位 50 岁女性，1 个月前出现下肢水肿和体重增加 5kg，1999 年 10 月因疑似肾病综合征入院。尿蛋白水平为每天 3.5g，尿高倍镜下红细胞 10-15 个，血清白蛋白水平 2.5g/dl。此外，胸部 X 线证实有胸腔积液积聚。肾活检结果显示肾小球系膜轻度增生，免疫荧光研究证实免疫球蛋白（Ig）A 和 C3 在系膜区沉积。电子显微镜观察到足突弥漫性稀疏和系膜区致密沉积物。给予 40mg/天泼尼松龙治疗有效，1 个月后蛋白尿转为阴性。由于这一病程，我们诊断为伴有轻度增生性 IgA 肾病的微小病变性肾病综合征。2000 年 11 月，肾病综合征复发，据信是由流感疫苗接种引起的，但增加激素治疗的反应良好，激素增加第 13 天蛋白尿消失。2001 年 5 月第二次复发，表现为激素抵抗性肾功能不全，选择性指数增加至 0.195。光镜显示肾小球局灶性硬化病变，免疫荧光研究显示系膜 IgA 和 C3 沉积减弱。这些发现提示微小病变性肾病综合征已转变为局灶节段性肾小球硬化，系膜 IgA 沉积通过免疫抑制治疗减少。此后，她的肾功能逐渐恶化，在肾病综合征第二次复发后 27 个月时进展至终末期肾衰竭。
