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特发性膜性肾病的不可预测性:一例病例报告

The Unpredictability of Idiopathic Membranous Nephropathy: An Illustrative Case Report.

作者信息

Levin Murray L, Ahya Shubhada

机构信息

Division of Nephrology/Hypertension, Department of Medicine, Northwestern University Feinberg School of Medicine and Northwestern Memorial Hospital, Chicago, Illinois, USA.

出版信息

Case Rep Nephrol Dial. 2018 Jun 1;8(2):103-106. doi: 10.1159/000489773. eCollection 2018 May-Aug.

DOI:10.1159/000489773
PMID:29928646
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6006632/
Abstract

The case of a female patient with primary membranous nephropathy is presented. She was treated with corticosteroids and chlorambucil after conservative therapy had failed and went into remission for 5 years. Her nephrotic syndrome recurred but did not respond to the same regimen. She had another complete remission after treatment with corticosteroids and cyclosporine, but the nephrosis recurred after 7 years. Again, she failed to respond with retreatment of steroids plus cyclosporine. She was treated with alternate-day steroid plus mycophenolate and, once again, had a complete remission. She was maintained on low-dose mycophenolate for 7 more years. The mycophenolate had to be discontinued because of cytomegalovirus colitis. Treatment with ganciclovir abolished the colitis. She is still in remission 10 years later. The case is discussed with regard to current knowledge of the immune pathogenesis of membranous nephropathy as well as the unknowns of the immunogenesis of the disease.

摘要

本文介绍了一位原发性膜性肾病女性患者的病例。保守治疗失败后,她接受了糖皮质激素和苯丁酸氮芥治疗,并缓解了5年。她的肾病综合征复发,但对相同治疗方案无反应。在接受糖皮质激素和环孢素治疗后,她再次完全缓解,但7年后肾病复发。同样,再次使用类固醇加环孢素治疗无效。她接受了隔日类固醇加霉酚酸酯治疗,再次完全缓解。她继续使用低剂量霉酚酸酯维持治疗7年多。由于巨细胞病毒性结肠炎,不得不停用霉酚酸酯。更昔洛韦治疗消除了结肠炎。10年后她仍处于缓解状态。结合目前对膜性肾病免疫发病机制的认识以及该疾病免疫发生的未知因素对该病例进行了讨论。

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