伊帕克潘首次成功治疗原发性免疫复合物-膜增生性肾小球肾炎患者:病例报告。

IF 0.7 Q4 UROLOGY & NEPHROLOGY Case Reports in Nephrology and Dialysis Pub Date : 2024-08-07 eCollection Date: 2024-01-01 DOI:10.1159/000540013
Simone Arnold, Manuela Nickler, Michael Dickenmann, Thomas Menter, Helmut Hopfer, Patricia Hirt-Minkowski
{"title":"伊帕克潘首次成功治疗原发性免疫复合物-膜增生性肾小球肾炎患者:病例报告。","authors":"Simone Arnold, Manuela Nickler, Michael Dickenmann, Thomas Menter, Helmut Hopfer, Patricia Hirt-Minkowski","doi":"10.1159/000540013","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Nowadays, there is insufficient evidence for the recommendation of management patients with a primary membranoproliferative glomerulonephritis (MPGN). A better understanding of the pathogenesis has led to the reclassification of primary MPGN and distinction into the two main entities of either primary immune complex-MPGN or C3 glomerulopathy. Both entities share overlapping pathophysiological features with complement alternative pathway (AP) dysregulation. Iptacopan is an oral inhibitor of the complement factor B that effectively blocks the complement AP.</p><p><strong>Case presentation: </strong>We report the first successful treatment of a 47-year-old man suffering from a primary immune complex-MPGN with iptacopan. So far established immunosuppressive therapies with prednisone and mycophenolate mofetil failed to control the current flare of the disease, mainly presenting with impaired kidney function and proteinuria within the nephrotic range. However, 3 months after starting the treatment with iptacopan urine protein-creatinine ratio decreased impressively to a level of 100-150 mg/mmol. Thereafter, low-level proteinuria and kidney function remained stable during follow-up. Do date, the treatment with iptacopan is continued as a monotherapy and is well tolerated.</p><p><strong>Conclusion: </strong>To the best of our knowledge, this is the first case report which suggests that iptacopan may be an interesting treatment option for primary immune complex-MPGN.</p>","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":null,"pages":null},"PeriodicalIF":0.7000,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11309753/pdf/","citationCount":"0","resultStr":"{\"title\":\"First Successful Treatment of a Patient with a Primary Immune Complex-Membranoproliferative Glomerulonephritis with Iptacopan: A Case Report.\",\"authors\":\"Simone Arnold, Manuela Nickler, Michael Dickenmann, Thomas Menter, Helmut Hopfer, Patricia Hirt-Minkowski\",\"doi\":\"10.1159/000540013\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Nowadays, there is insufficient evidence for the recommendation of management patients with a primary membranoproliferative glomerulonephritis (MPGN). A better understanding of the pathogenesis has led to the reclassification of primary MPGN and distinction into the two main entities of either primary immune complex-MPGN or C3 glomerulopathy. Both entities share overlapping pathophysiological features with complement alternative pathway (AP) dysregulation. Iptacopan is an oral inhibitor of the complement factor B that effectively blocks the complement AP.</p><p><strong>Case presentation: </strong>We report the first successful treatment of a 47-year-old man suffering from a primary immune complex-MPGN with iptacopan. So far established immunosuppressive therapies with prednisone and mycophenolate mofetil failed to control the current flare of the disease, mainly presenting with impaired kidney function and proteinuria within the nephrotic range. However, 3 months after starting the treatment with iptacopan urine protein-creatinine ratio decreased impressively to a level of 100-150 mg/mmol. Thereafter, low-level proteinuria and kidney function remained stable during follow-up. Do date, the treatment with iptacopan is continued as a monotherapy and is well tolerated.</p><p><strong>Conclusion: </strong>To the best of our knowledge, this is the first case report which suggests that iptacopan may be an interesting treatment option for primary immune complex-MPGN.</p>\",\"PeriodicalId\":9599,\"journal\":{\"name\":\"Case Reports in Nephrology and Dialysis\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2024-08-07\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11309753/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Nephrology and Dialysis\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1159/000540013\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Nephrology and Dialysis","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000540013","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0

摘要

简介目前,还没有足够的证据对原发性膜增生性肾小球肾炎(MPGN)患者的治疗提出建议。随着对发病机理的深入了解,人们对原发性膜增生性肾小球肾炎进行了重新分类,并将其分为原发性免疫复合物-膜增生性肾小球肾炎或 C3 肾小球病两大类。这两种疾病都具有补体替代途径(AP)失调的重叠病理生理特征。Iptacopan是一种口服补体因子B抑制剂,能有效阻断补体AP:病例介绍:我们报告了伊帕可潘首次成功治疗一名 47 岁男性原发性免疫复合物-MPGN 患者的病例。迄今为止,使用泼尼松和霉酚酸酯进行的免疫抑制治疗未能控制目前的病情发作,主要表现为肾功能受损和肾病范围内的蛋白尿。然而,在开始使用依帕可潘治疗 3 个月后,尿蛋白-肌酐比值显著下降至 100-150 毫克/毫摩尔的水平。此后,低水平蛋白尿和肾功能在随访期间保持稳定。迄今为止,伊帕可潘作为一种单一疗法仍在继续使用,且耐受性良好:据我们所知,这是第一份病例报告,它表明依帕可潘可能是治疗原发性免疫复合物-MPGN 的一种有趣选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
First Successful Treatment of a Patient with a Primary Immune Complex-Membranoproliferative Glomerulonephritis with Iptacopan: A Case Report.

Introduction: Nowadays, there is insufficient evidence for the recommendation of management patients with a primary membranoproliferative glomerulonephritis (MPGN). A better understanding of the pathogenesis has led to the reclassification of primary MPGN and distinction into the two main entities of either primary immune complex-MPGN or C3 glomerulopathy. Both entities share overlapping pathophysiological features with complement alternative pathway (AP) dysregulation. Iptacopan is an oral inhibitor of the complement factor B that effectively blocks the complement AP.

Case presentation: We report the first successful treatment of a 47-year-old man suffering from a primary immune complex-MPGN with iptacopan. So far established immunosuppressive therapies with prednisone and mycophenolate mofetil failed to control the current flare of the disease, mainly presenting with impaired kidney function and proteinuria within the nephrotic range. However, 3 months after starting the treatment with iptacopan urine protein-creatinine ratio decreased impressively to a level of 100-150 mg/mmol. Thereafter, low-level proteinuria and kidney function remained stable during follow-up. Do date, the treatment with iptacopan is continued as a monotherapy and is well tolerated.

Conclusion: To the best of our knowledge, this is the first case report which suggests that iptacopan may be an interesting treatment option for primary immune complex-MPGN.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
CiteScore
1.20
自引率
0.00%
发文量
36
审稿时长
10 weeks
期刊介绍: This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of nephrology and dialysis, including genetic susceptibility, clinical presentation, diagnosis, treatment or prevention, toxicities of therapy, critical care, supportive care, quality-of-life and survival issues. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed.
期刊最新文献
Successful Repeated Use of a Pathogen Adsorbing Biomimetic Device for the Adjunct Treatment of a SARS-CoV-2 Reinfection and Subsequent Infections with Different Multiresistant Bacteria. Recurrent Fibrillary Glomerulonephritis Secondary to Chronic Lymphocytic Leukemia: Remission of Kidney Disease with Ibrutinib. Long-Term Observation of Focal Segmental Glomerulosclerosis after Treatment of Renal Parenchymal Malakoplakia: A Case Report. JC Polyomavirus Nephropathy: A Rare Complication Late after Kidney Transplantation. First Successful Treatment of a Patient with a Primary Immune Complex-Membranoproliferative Glomerulonephritis with Iptacopan: A Case Report.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1