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
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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。
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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.

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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
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