Eculizumab treatment for rescue of renal function in IgA nephropathy
Eculizumab treatment for rescue of renal function in IgA nephropathy
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DOI：
10.1007/s00467-014-2863-y
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发表时间：
2014-11-01
影响因子：
3
通讯作者：
Karpman, Diana
中科院分区：
文献类型：
--
作者：
Rosenblad, Therese;Rebetz, Johan;Karpman, Diana
免疫球蛋白A（伊加）肾病是一种慢性肾小球肾炎，肾小球中IgA 1、C3和C5 b-9的过度沉积可能导致肾功能衰竭。我们描述了一个青少年的临床过程，尽管免疫抑制治疗，但疾病进展迅速，导致肾功能衰竭。由于难治性疾病，患者接受依库珠单抗（抗C5）治疗3个月，试图挽救肾功能。治疗导致临床改善，肾小球滤过率稳定，蛋白尿减少。停止治疗导致肾功能迅速恶化。随后给予单剂量依库珠单抗，再次暂时降低肌酐水平。早期开始依库珠单抗治疗进展性伊加肾病患者可能通过阻断补体介导的肾脏炎症产生有益效果。
Immunoglobulin A (IgA) nephropathy is a chronic glomerulonephritis with excessive glomerular deposition of IgA1, C3 and C5b-9, which may lead to renal failure.We describe the clinical course of an adolescent with rapidly progressive disease leading to renal failure in spite of immunosuppressive treatment. Due to refractory disease the patient was treated with eculizumab (anti-C5) for 3 months in an attempt to rescue renal function. Treatment led to clinical improvement with stabilization of the glomerular filtration rate and reduced proteinuria. Discontinuation of treatment led to a rapid deterioration of renal function. This was followed by a single dose of eculizumab, which again reduced creatinine levels temporarily.Early initiation of eculizumab therapy in patients with progressive IgA nephropathy may have a beneficial effect by blocking complement-mediated renal inflammation.