抗磷脂酶A2受体抗体与特发性膜性肾病培训讲学.pptVIP

抗磷脂酶A2受体抗体与特发性膜性肾病 秦卫松 南京军区南京总医院 全军肾脏病研究所 全军肾脏病重点实验室 Beck Jr LH, et al. N Engl J Med 2009; 361: 11 Western Blotting of Glomerular Proteins with Serum from Patients with IMN. Glassock RJ. N Engl J Med,2009,361: 81 病例选择 特发性膜性肾病：60例 肾活检时尿蛋白定量3.5g/24hr 经肾活检病理明确诊断为肾小球膜性病变， 光镜观察无内皮或系膜细胞增殖，无内皮下嗜复红物沉积， 免疫荧光以IgG、C3为主, C1q阴性； 自身抗体检测阴性，无乙肝、丙肝感染、肿瘤病史，无重金属、有机溶剂接触史； 特发性膜性肾病治疗后缓解的病例：21例 病例选择 V型狼疮性肾炎：20例 系统性红斑狼疮、狼疮性肾炎诊断明确； 病理上以V型（膜性）病变为主，免疫荧光以颗粒状上皮侧沉积为主，无明显系膜或内皮细胞增殖； 肾活检时尿蛋白定量3.5g/24hr，尿沉渣红细胞100万/ml。 乙肝相关膜性肾病：16例 经肾活检病理明确诊断为肾小球膜性病变； 乙肝两对半检测显示大三阳和/或HBV-DNA阳性； 肾组织乙肝抗原染色阳性(其中HBeAg必须阳性)； 经抗病毒治疗后患者尿蛋白部分缓解或完全缓解； 肾活检时尿蛋白定量3.5g/24hr。 肿瘤相关膜性肾病：10例 肾活检时尿蛋白定量3.5g/24hr 从肾小球提取蛋白抗原 SDS(Non-reduced) 转印至硝酸纤维素膜 与患者血清孵育（1：25） 一抗，二抗，显色 实验方法 研究结果 Detection of anti-PLA2R in idiopathic MN Recognition of idiopathic MN serum with deglycosylated PLA2R The prevalence of anti-PLA2R in idiopathic MN and secondary MN n Anti-PLA2R % Idiopathic MN 60 49 81.7% Lupus MN 20 1 5.0% HBV-MN 16 1 6.3% Tumor-MN 10 3 30% Table Anti-PLA2R in Chinese patients with membranous nephropathy There was an absence of immunologic disease activity at the time when their serum was sampled, despite proteinuria. Why remain 11 IMN patients negative? Low titer of anti-PLA2R in negative idiopathic MN Low titer of anti-PLA2R existed in 10 of remaining 11 negative IMN. PC: a MN serum that was positive under standard conditions diluted to 1:1000. HC, Healthy control, serum from healthy adult (1:10). Association of anti-PLA2R with disease activity Anti-PLA2R in IMN and IMN with complete remission 81.7% 19.0% 结 论 PLA2R 是中国人特发性膜性肾病的重要靶抗原。 抗PLA2R抗体可以作为特发性膜性肾病诊断和疗效判断的特异标志物。 致 谢 刘志红院士 David J. Salant Laurence H. Beck, Jr. 陈朝红 曾彩虹 左科 IgG4 in a glomerulus of a patient with tumor-MN but without circulating anti-PLA2R antibodies. Bright IgG4 in a glomerulus of a patient with tumor-MN and circulating anti-PLA2R antibodies. IgG4 deposition in glomeruli of tumor-MN. All three patients with Anti-PLA2R : persistent or relap