Eculizumab treatment for rescue of renal function in IgA nephropathy

被引:113
作者
Rosenblad, Therese [1 ]
Rebetz, Johan [2 ]
Johansson, Martin [3 ,4 ]
Bekassy, Zivile [1 ]
Sartz, Lisa [1 ]
Karpman, Diana [1 ,2 ]
机构
[1] Skane Univ Hosp, Dept Pediat & Adolescent Med, Sect Pediat Nephrol, Lund, Sweden
[2] Lund Univ, Dept Pediat, S-22185 Lund, Sweden
[3] Skane Univ Hosp, Dept Pathol, Malmo, Sweden
[4] Lund Univ, Ctr Mol Pathol, Dept Lab Med Malmo, Malmo, Sweden
基金
瑞典研究理事会;
关键词
IgA nephropathy; Complement; Eculizumab; IMMUNOGLOBULIN-A NEPHROPATHY; FACTOR-H; GLOMERULAR DEPOSITION; COMPLEMENT; ACTIVATION; GLOMERULONEPHRITIS; PROTEINS; C5B-9;
D O I
10.1007/s00467-014-2863-y
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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.
引用
收藏
页码:2225 / 2228
页数:4
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