PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■如今，对于原发性膜增生性肾小球肾炎(MPGN)患者的治疗建议证据不足.对发病机理的更好理解导致了原发性MPGN的重新分类，并区分为原发性免疫复合物-MPGN或C3肾小球病的两个主要实体。两种实体都具有重叠的病理生理学特征，具有补体替代途径（AP）失调。Iptacopan是补体因子B的口服抑制剂，可有效阻断补体AP。
■我们报道了一名47岁的男性首次成功治疗，该男性患有伊托科潘的原发性免疫复合物-MPGN。到目前为止，使用泼尼松和霉酚酸酯建立的免疫抑制疗法未能控制当前的疾病发作，主要表现为肾功能受损和肾病范围内的蛋白尿。然而,在开始用伊塔科班治疗3个月后，尿蛋白-肌酐比率令人印象深刻地降低至100-150mg/mmol的水平。此后,低水平蛋白尿和肾功能在随访期间保持稳定.Dodate,伊塔科班作为单一疗法继续治疗,且耐受性良好.
■据我们所知,这是首例病例报告,提示伊塔科班可能是原发性免疫复合物-MPGN的一个有趣的治疗选择.
■我们报道了一名47岁的男性首次成功治疗，该男性患有伊托科潘的原发性免疫复合物-MPGN。到目前为止，使用泼尼松和霉酚酸酯建立的免疫抑制疗法未能控制当前的疾病发作，主要表现为肾功能受损和肾病范围内的蛋白尿。然而,在开始用伊塔科班治疗3个月后，尿蛋白-肌酐比率令人印象深刻地降低至100-150mg/mmol的水平。此后,低水平蛋白尿和肾功能在随访期间保持稳定.Dodate,伊塔科班作为单一疗法继续治疗,且耐受性良好.
■据我们所知,这是首例病例报告,提示伊塔科班可能是原发性免疫复合物-MPGN的一个有趣的治疗选择.
