B cell phenotype in pediatric idiopathic nephrotic syndrome
B cell phenotype in pediatric idiopathic nephrotic syndrome
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DOI：
10.1007/s00467-018-4095-z
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发表时间：
2019-01-01
影响因子：
3
通讯作者：
Vivarelli, Marina
中科院分区：
文献类型：
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作者：
Colucci, Manuela;Carsetti, Rita;Vivarelli, Marina
背景B细胞在非遗传性肾病综合征中的致病作用已被利妥昔单抗（一种B细胞耗竭抗体）在维持长期缓解中的疗效所证实。然而，关于肾病综合征患者B细胞稳态的信息很少。MethodsWe回顾性分析了107例激素敏感和6例遗传性激素耐药肾病综合征患儿不同B细胞亚群的分布，并与年龄和性别匹配的对照组进行比较。与对照组相比，显示出总的、过渡的、记忆的和转换的记忆B细胞的水平显著增加。口服免疫抑制剂强烈影响27例复发患者和29例缓解患者的过渡期和成熟B细胞水平，而记忆B细胞在复发期间显著高于对照组，尽管免疫抑制剂治疗，仅在缓解患者中正常化。遗传形式的类固醇耐药型肾病综合征的儿童在B细胞配置文件从controls.ConclusionsOur研究表明，记忆B细胞，比其他B细胞亚群，增加，似乎是类固醇敏感型肾病综合征儿科患者的致病相关。
BackgroundA pathogenic role of B cells in non-genetic nephrotic syndrome has been suggested by the efficacy of rituximab, a B cell depleting antibody, in maintaining a prolonged remission. However, little information is available on B cell homeostasis in nephrotic syndrome patients.MethodsWe retrospectively analyzed by flow cytometry the distribution of different B cell subpopulations in 107 steroid-sensitive and in 6 genetic steroid-resistant nephrotic syndrome pediatric patients, compared with age- and sex-matched controls.ResultsFifty-one steroid-sensitive patients at disease onset, before starting immunosuppression, presented significantly increased levels of total, transitional, memory, and switched memory B cells compared to controls. Oral immunosuppression strongly affected transitional and mature B cell levels in 27 patients in relapse and also in 29 patients in remission, whereas memory B cells were significantly higher compared to controls during relapse, despite the immunosuppressive treatment, and were normalized only in patients in remission. Children with genetic forms of steroid-resistant nephrotic syndrome presented no differences in B cell profile from controls.ConclusionsOur study indicates that memory B cells, more than other B cell subsets, are increased and appear to be pathogenically relevant in steroid-sensitive nephrotic syndrome pediatric patients.