儿童特发性膜性肾病用利妥昔单抗治疗：两例报告。
Idiopathic membranous nephropathy in children treated with rituximab: report of two cases.
机构信息
Division of Nephrology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA.
Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, USA.
出版信息
Pediatr Nephrol. 2018 Jun;33(6):1089-1092. doi: 10.1007/s00467-018-3923-5. Epub 2018 Mar 15.
BACKGROUND
Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents.
CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab. Both patients received two doses of rituximab and responded with remission. In addition, anti-PLA2R antibody levels normalized and/or significantly improved.
CONCLUSIONS
Rituximab seems to be a safe and effective treatment option in children with idiopathic membranous nephropathy due to anti-PLA2R. Further studies are needed to evaluate this effectiveness.
背景
特发性膜性肾病是儿童肾病综合征的一种不常见病因，可能带来治疗挑战。目前的治疗选择包括类固醇和环磷酰胺、环孢素或霉酚酸酯，这些治疗需要长期进行，并且相关的副作用可能导致儿童（尤其是青少年）不依从治疗。
病例诊断
我们报告了两名特发性膜性肾病青少年患者，他们均有肾病范围蛋白尿和升高的抗磷脂酶 A2 受体水平，在接受类固醇治疗后未缓解，后来接受了利妥昔单抗治疗。两名患者均接受了两剂利妥昔单抗治疗，缓解效果显著。此外，抗-PLA2R 抗体水平正常化和/或显著改善。
结论
利妥昔单抗似乎是特发性膜性肾病（抗-PLA2R 型）儿童的一种安全有效的治疗选择。需要进一步的研究来评估这种有效性。
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