Treatment of Idiopathic Membranous Nephropathy (IMN)

M. Soler, M. Pino, A. P. Fernández, Llenalia García, M. Ruiz, C. Ramírez-Tortosa
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Abstract

We present a 59-year-old patient with type 2 diabetes mellitus and massive nephrotic syndrome (anasarca) and biochemical syndrome. The renal biopsy showed a membranous nephropathy (MN). In the blood analysis the patient presented antibodies against M-type phospholipase A2 receptor (anti-PLA2R) positive at a very high titer. Given the existence of idiopathic membranous nephropathy (IMN), treatment was started with a modified Ponticelli regimen, with no response, requiring periodic ultrafiltration sessions. Rituximab induces nephrotic syndrome (NS) remission in two-thirds of patients with IMN, even after other treatments have failed. We proposed treatment with rituximab based on published evidence. In IMN, the presence of M-type anti-receptor antibodies of A2 phospholipase is considered highly specific to idiopathic forms, but the presence of such antibodies has not been shown to be associated with a particular clinical profile. Assessing circulating antiPLA2R autoantibodies and proteinuria may help in monitoring disease activity and guiding personalized rituximab therapy in nephrotic patients with IMN.
特发性膜性肾病的治疗
我们报告一位59岁的2型糖尿病合并大量肾病综合征(anasarca)和生化综合征的患者。肾活检显示膜性肾病(MN)。在血液分析中,患者呈m型磷脂酶A2受体抗体(抗pla2r)阳性,滴度非常高。考虑到特发性膜性肾病(IMN)的存在,治疗开始时采用改良的Ponticelli方案,没有反应,需要定期超滤。利妥昔单抗在三分之二的IMN患者中诱导肾病综合征(NS)缓解,即使在其他治疗失败后也是如此。根据已发表的证据,我们建议使用利妥昔单抗治疗。在IMN中,A2磷脂酶的m型抗受体抗体的存在被认为是特发性形式的高度特异性,但这种抗体的存在并未显示与特定的临床特征相关。评估循环抗pla2r自身抗体和蛋白尿可能有助于监测疾病活动性,指导IMN肾病患者的个性化利妥昔单抗治疗。
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