特发性膜性肾小球的B淋巴细胞耗竭:来自延长随访的患者的结果。

Nephron Extra Pub Date : 2013-01-01 Epub Date: 2013-01-05 DOI:10.1159/000345487
Sophia Lionaki, Smaragdi Marinaki, Lydia Nakopoulou, Chrysanthi Skalioti, Aliki Iniotaki, Petros P Sfikakis, Costas Siamopoulos, John Boletis
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引用次数: 4

摘要

目的:评估特发性膜性肾小球病(MGN)患者暂时减少B淋巴细胞的长期治疗效果,并寻找反应的潜在预测因素。患者和方法:纳入的患者在没有继发原因的情况下被活检证实为MGN。估计肾小球滤过率应大于30ml /min/1.73 m(2), 24小时蛋白尿≥3g /天。在研究开始前一年接受过环孢素或细胞毒性药物治疗的患者被排除在外。使用利妥昔单抗(rituximab)实现B细胞的清除,连续4周静脉注射利妥昔单抗。部分缓解定义为蛋白尿减少>50%,绝对蛋白尿减少50%,绝对蛋白质排泄。结果:研究了12例患者(4女8男),平均年龄51.3岁。没有观察到主要的不良反应。在中位随访48个月期间,11/12(91.6%)例患者获得缓解[7/12(58.3%)完全缓解,4/12(33.3%)部分缓解],1例患者对治疗无反应。治疗12个月后,68.8% (p = 0.003)的病例部分缓解,28.4%的病例完全缓解。淋巴细胞亚群的测量没有发现任何变化,除了B细胞耗竭。诊断活检中每mm(3)肾组织捕获的B细胞浸润与随后的反应无关。结论:在12例特发性MGN患者中,B细胞的消耗具有良好的耐受性,并产生了显著且持久的缓解率。
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Depletion of B lymphocytes in idiopathic membranous glomerulopathy: results from patients with extended follow-up.

Aims: To assess the long-term therapeutic benefit of temporary depletion of B lymphocytes in patients with idiopathic membranous glomerulopathy (MGN) and search for potential predictors of response.

Patients and methods: The patients included had been diagnosed with biopsy-proven MGN in the absence of secondary causes. Estimated glomerular filtration rate should be above 30 ml/min/1.73 m(2) and 24-hour proteinuria 3 g/day or more. Patients who had been treated with cyclosporine or cytotoxic agents the year prior to study entry were excluded. Depletion of B cells was achieved with rituximab, which was administered intravenously for 4 consecutive weeks. Partial remission was defined as a >50% decrease in proteinuria with absolute proteinuria <3 g/day, while complete remission was defined as a >50% decrease in proteinuria and an absolute protein excretion <0.3 g/day.

Results: Twelve patients were studied (4 females/8 males) with a mean age of 51.3 years. No major adverse effects were observed. During a median follow-up time of 48 months, 11/12 (91.6%) patients achieved remission [7/12 (58.3%) complete remission and 4/12 (33.3%) partial remission], while 1 patient did not respond to therapy. Twelve months after therapy, 68.8% (p = 0.003) of cases had achieved partial and 28.4% complete remission. Measurements of lymphocyte subpopulations did not reveal any changes except for the B cell depletion. B cell infiltrates captured per mm(3) of renal tissue in the diagnostic biopsy did not correlate with subsequent response.

Conclusion: Depletion of B cells in idiopathic MGN was well tolerated and resulted in significant and long-lasting response rates in a series of 12 patients.

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期刊介绍: An open-access subjournal to Nephron. ''Nephron EXTRA'' publishes additional high-quality articles that cannot be published in the main journal ''Nephron'' due to space limitations.
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