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纯合子FCGR3A - 158F突变与利妥昔单抗治疗后B细胞耗竭延迟相关,但在一名难治性狼疮性肾炎患者中疗效得以保留。

Homozygous FCGR3A-158F mutation is associated with delayed B-cell depletion following rituximab but with preserved efficacy in a patient with refractory lupus nephritis.

作者信息

Seret Guillaume, Hanrotel Catherine, Bendaoud Boutahar, Le Meur Yannick, Renaudineau Yves

机构信息

EA2216 Immunology and Pathology, European University of Brittany, Brest, France; Laboratory of Immunology, Brest University Medical School, CHRU Morvan,Brest, France; Unit of Nephrology, Brest University Medical School, CHRU Cavale Blanche, Brest, France.

Unit of Nephrology , Brest University Medical School, CHRU Cavale Blanche , Brest , France.

出版信息

Clin Kidney J. 2013 Feb;6(1):74-76. doi: 10.1093/ckj/sfs162. Epub 2012 Dec 20.

DOI:10.1093/ckj/sfs162
PMID:27818754
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5094396/
Abstract

Rituximab (RTX), an anti-CD20 monoclonal antibody, has shown promising results in a small group of systemic lupus erythematosus (SLE) patients treated for lupus nephritis (LN). However, such observations were not confirmed in the double-blind LUNAR study. Accordingly, the factors associated with the clinical response remain to be characterized. We report the case of a young woman with known LN successfully re-treated with RTX and steroids and homozygous for the low-affinity FCG3RA 158F genotype. Although B-cell depletion was delayed, complete remission with anti-DNA antibody negativity and proteinuria normalization were maintained for 5 years. The implications for disease pathogenesis and clinical monitoring are discussed.

摘要

利妥昔单抗(RTX)是一种抗CD20单克隆抗体,在一小部分接受狼疮性肾炎(LN)治疗的系统性红斑狼疮(SLE)患者中显示出了有前景的结果。然而,在双盲的LUNAR研究中并未证实这些观察结果。因此,与临床反应相关的因素仍有待明确。我们报告了一例已知患有LN的年轻女性患者,成功接受了RTX和类固醇再次治疗,且为低亲和力FCG3RA 158F基因型的纯合子。尽管B细胞耗竭延迟,但抗DNA抗体转阴和蛋白尿正常化的完全缓解状态维持了5年。文中讨论了其对疾病发病机制和临床监测的意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7be1/5094396/ee1fca7e6034/sfs16201.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7be1/5094396/ee1fca7e6034/sfs16201.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7be1/5094396/ee1fca7e6034/sfs16201.jpg

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