Ogura Yoshiyasu, Yabushita Sayaka, Aihara Hideki, Tsukada Hiroyuki, Hashiba Toyohiro, Furuse Satoshi, Fujii Akiko, Ueda Yoshihiko, Mise Naobumi
Department of Nephrology, Division of Internal Medicine, Mitsui Memorial Hospital, 1 Kanda-izumi-cho, Chiyoda-ku, Tokyo, 101-8643, Japan.
Department of Pathology, Saitama Medical Center, Dokkyo Medical University, 2-1-50 Minamikoshigaya, Koshigaya city, Saitama, 343-8555, Japan.
CEN Case Rep. 2022 May;11(2):208-215. doi: 10.1007/s13730-021-00653-3. Epub 2021 Oct 10.
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) generally has a poor prognosis and the consensus is that it needs to be treated with clone-directed therapy. However, the prognosis of PGNMID is heterogenous and some cases have been successfully treated using other therapeutic strategies. We herein report a case of PGNMID that responded favorably to steroids without clone-directed therapy. An 18-year-old woman was referred to a nephrologist with proteinuria detected in an annual health check-up. Over a 3-year period, the concentration of creatinine (Cr) increased from 0.76 to 1.00 mg/dL and proteinuria from 0.35 to 1.9 g/g Cr. Monoclonal gammopathies were not detected in her serum or urine. Based on the findings of kidney biopsy at the age of 21 years, the patient was diagnosed with proliferative glomerulonephritis with monoclonal IgG1-kappa deposits. The histological feature was mesangial proliferative glomerulonephritis with advanced glomerulosclerosis, which is a rare presentation of PGNMID. Intravenous methylprednisolone pulse therapy was initiated, followed by oral prednisolone at a dose of 30 mg daily. One year later, a second kidney biopsy revealed a significant decrease in mesangial deposits of IgG1-kappa. Prednisolone was gradually tapered and discontinued 2 years after the first kidney biopsy. At the time of prednisolone withdrawal, urinalysis showed proteinuria of 0.2 g/g Cr without hematuria. Kidney function remained stable throughout the treatment period.
伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎（PGNMID）通常预后较差，目前的共识是需要采用针对克隆的疗法进行治疗。然而，PGNMID的预后存在异质性，一些病例已通过其他治疗策略成功治愈。我们在此报告一例PGNMID患者，其在未接受针对克隆的治疗情况下对类固醇治疗反应良好。一名18岁女性在年度健康检查中被检测出蛋白尿，随后被转诊至肾病科医生处。在3年时间里，肌酐（Cr）浓度从0.76mg/dL升至1.00mg/dL，蛋白尿从0.35g/g Cr增至1.9g/g Cr。其血清和尿液中均未检测到单克隆丙种球蛋白病。根据21岁时肾脏活检的结果，该患者被诊断为伴有单克隆IgG1-κ沉积的增殖性肾小球肾炎。组织学特征为系膜增生性肾小球肾炎伴晚期肾小球硬化，这是PGNMID的一种罕见表现。开始静脉注射甲泼尼龙冲击治疗，随后口服泼尼松龙，剂量为每日30mg。一年后，第二次肾脏活检显示IgG1-κ的系膜沉积物显著减少。泼尼松龙逐渐减量，并在第一次肾脏活检后2年停药。在停用泼尼松龙时，尿液分析显示蛋白尿为0.2g/g Cr，无血尿。在整个治疗期间，肾功能保持稳定。
