[The 493th case: recurrent edema of bilateral lower extremeties]

Zhonghua Nei Ke Za Zhi. 2022 Mar 1;61(3):355-358. doi: 10.3760/cma.j.cn112138-20210603-00393.
[Article in Chinese]

Abstract

A 43-year-old male patient with onset of edema caused by nephrotic proteinuria and low titer of anti-M type phospholipase-A2-receptor (PLA2R) antibody was diagnosed as idiopathic membranous nephropathy by renal biopsy. Administrated with prednisone 40 mg once a day and cyclosporine 100 mg twice a day as front-line regimen, the patient relapsed after transient partial remission. When treatment was combined with cyclophosphamide 100 mg once a day, the 24-hour total urine protein and titer of anti-PLA2R antibody were even elevated. Therefore, the patient received rituximab 1 g intravenously in April 2019, October 2019 and October 2020 respectively. CD19 positive B lymphocytes in peripheral blood were eliminated from 71/μl to zero. Immunosuppressants and corticosteroids were withdrawn successively. On the last follow-up in November 2020, the anti-PLA2R antibody was negative, and the 24-hour total urine protein and serum albumin was 4.4 g and 34 g/L, respectively. This case suggested the potential efficacy of rituximab for refractory membranous nephropathy. Further studies should explore whether the titer of anti-PLA2R antibody indicates the dose of rituximab.

患者男性,43岁。以水肿起病,查24h尿蛋白定量4.66 g,抗M型磷脂酶A2受体(PLA2R)抗体水平25.3 EU/ml,肾脏穿刺病理确诊特发性膜性肾病(MN)。初期口服泼尼松40 mg每日1次,环孢素A 100 mg每日2次治疗,部分缓解后复发,联合环磷酰胺100 mg每日1次口服无效,监测24h尿蛋白定量升至10.8 g,抗PLA2R抗体水平升至496 RU/ml,CD19+B细胞计数71个/μl,遂分别予3次利妥昔单抗1 g静脉输液治疗,并先后停用环孢素A、环磷酰胺及泼尼松。末次随访CD19+B细胞0个/μl,抗PLA2R抗体阴性,血白蛋白34 g/L,24 h尿蛋白定量4.4 g。本例患者提示了利妥昔单抗在难治性膜性肾病中的治疗效果,以CD19+B细胞计数或抗PLA2R抗体水平作为再次使用利妥昔单抗治疗的指征,需进一步思考。.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies*
  • Cyclophosphamide / therapeutic use
  • Edema
  • Glomerulonephritis, Membranous*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Receptors, Phospholipase A2

Substances

  • Autoantibodies
  • Immunosuppressive Agents
  • Receptors, Phospholipase A2
  • Cyclophosphamide