Bharati Joyita, Tiewsoh Karalanglin, Kumar Ashwani, Nada Ritambhra, Rathi Manish, Gupta Krishan Lal, Kohli Harbir Singh, Jha Vivekananda, Ramachandran Raja
Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Clin Kidney J. 2018 Dec 24;12(4):483-487. doi: 10.1093/ckj/sfy127. eCollection 2019 Aug.
C3 glomerulopathy (C3G) is a heterogeneous disease caused by alternative complement pathway abnormalities without any standardized treatment. An immunosuppressive agent, mycophenolate mofetil (MMF), has been recently shown to be useful in treating C3G, mainly in studies from the west. We report the clinical outcome of 17 Indian C3G patients treated with MMF with or without steroids.
The clinical and histology details of the C3G patients treated with MMF for at least 6 months with a follow-up of at least 12 months were retrieved from the medical records of our center.
The median serum creatinine and proteinuria at presentation were 0.8 mg/dL and 3.7 g/day, respectively, with the majority (88.2%) presenting as nephrotic syndrome. The mean dose of MMF was 1.65 (±0.56) g/day, and the median duration of MMF therapy was 18 months. Two-thirds (64%) of the patients responded to the treatment, with complete remission in 4 (23%) and partial remission in 7 (41%) (median time: 9 months). Three patients progressed to end-stage renal disease (ESRD) on follow-up. Of the three patients, one (33%) had an initial response in proteinuria to MMF but did not respond after a relapse and subsequently progressed to ESRD and two (67%) other patients were nonresponsive to MMF from the start of the therapy.
Despite a small sample size and lack of a control arm, this study describes the effectiveness of MMF in treating C3G patients from Asia and forms a basis for future randomized trials.
C3肾小球病(C3G)是一种由替代补体途径异常引起的异质性疾病,目前尚无标准化治疗方法。免疫抑制剂霉酚酸酯(MMF)最近已被证明对治疗C3G有效,主要是在西方的研究中。我们报告了17例接受MMF联合或不联合类固醇治疗的印度C3G患者的临床结果。
从我们中心的病历中检索接受MMF治疗至少6个月且随访至少12个月的C3G患者的临床和组织学细节。
就诊时血清肌酐和蛋白尿的中位数分别为0.8mg/dL和3.7g/天,大多数患者(88.2%)表现为肾病综合征。MMF的平均剂量为1.65(±0.56)g/天,MMF治疗的中位持续时间为18个月。三分之二(64%)的患者对治疗有反应,4例(23%)完全缓解,7例(41%)部分缓解(中位时间:9个月)。3例患者在随访中进展为终末期肾病(ESRD)。在这3例患者中,1例(33%)最初对MMF治疗蛋白尿有反应,但复发后无反应,随后进展为ESRD,另外2例(67%)患者从治疗开始就对MMF无反应。
尽管样本量小且缺乏对照组,但本研究描述了MMF治疗亚洲C3G患者的有效性,并为未来的随机试验奠定了基础。