Abstract：
BACKGROUND: This study aimed to investigate the role of neurofascin186 (NF186) in the pathogenesis of the concurrent focal segmental glomerulosclerosis (FSGS) in CIDP-like autoimmune nodopathy patients.
METHODS: We presented a case of CIDP-like autoimmune nodopathy complicated with FSGS. We measured NF186 antibodies by cell-binding assay (CBA) method. We performed immunofluorescence analysis in the renal cryosection samples from a patient with minimal nephropathy with rabbit anti-NF186 antibody or NF186 antibody positive human serum. Then we performed western blotting of recombinant NF186 protein and component of NF186 including Ig and FNIII domains incubating with human serum and corresponding rabbit polyclonal antibody. Cases of CIDP complicated with FSGS were searched form PubMed and reviewed.
RESULTS: We reported a 66-year-old Chinese woman with CIDP-like autoimmune nodopathy and concurrent FSGS. Her NF186 antibody was positive. The fluorescent signal for NF186 was detected in the renal tissue sections of the patient with minimal nephropathy. The staining for NF186 matched the podocyte spatially. In western blotting analysis, patients had antibodies in their serum recognizing the NF186 protein and their antibodies recognized the Ig domain of NF186. 3 cases of CIDP-like autoimmune nodopathy with positive NF186 antibody and FSGS have been reported. All these patients were responsive to corticosteroids rather than the intravenous immunoglobulin, in terms of both the neuropathy and renal disease.
CONCLUSIONS: NF186 was probably a targeted antigen in the pathogenesis of concurrent FSGS in CIDP-like autoimmune nodopathy with positive NF186 antibody. CIDP-like autoimmune nodopathy with positive NF186 antibody and FSGS is a rare entity, which may be responsive to corticosteroids combined with immunosuppressant.
METHODS: We presented a case of CIDP-like autoimmune nodopathy complicated with FSGS. We measured NF186 antibodies by cell-binding assay (CBA) method. We performed immunofluorescence analysis in the renal cryosection samples from a patient with minimal nephropathy with rabbit anti-NF186 antibody or NF186 antibody positive human serum. Then we performed western blotting of recombinant NF186 protein and component of NF186 including Ig and FNIII domains incubating with human serum and corresponding rabbit polyclonal antibody. Cases of CIDP complicated with FSGS were searched form PubMed and reviewed.
RESULTS: We reported a 66-year-old Chinese woman with CIDP-like autoimmune nodopathy and concurrent FSGS. Her NF186 antibody was positive. The fluorescent signal for NF186 was detected in the renal tissue sections of the patient with minimal nephropathy. The staining for NF186 matched the podocyte spatially. In western blotting analysis, patients had antibodies in their serum recognizing the NF186 protein and their antibodies recognized the Ig domain of NF186. 3 cases of CIDP-like autoimmune nodopathy with positive NF186 antibody and FSGS have been reported. All these patients were responsive to corticosteroids rather than the intravenous immunoglobulin, in terms of both the neuropathy and renal disease.
CONCLUSIONS: NF186 was probably a targeted antigen in the pathogenesis of concurrent FSGS in CIDP-like autoimmune nodopathy with positive NF186 antibody. CIDP-like autoimmune nodopathy with positive NF186 antibody and FSGS is a rare entity, which may be responsive to corticosteroids combined with immunosuppressant.
摘要：
背景：本研究旨在探讨神经束186（NF186）在CIDP样自身免疫性神经病变患者并发局灶性节段肾小球硬化（FSGS）发病机制中的作用。
方法：我们介绍了一例伴有FSGS的CI-DP样自身免疫性神经病变。我们通过细胞结合测定(CBA)方法测量NF186抗体。我们使用兔抗NF186抗体或NF186抗体阳性人血清对患有轻度肾病的患者的肾冷冻切片样品进行了免疫荧光分析。然后我们对与人血清和相应的兔多克隆抗体一起孵育的重组NF186蛋白和包括Ig和FNIII结构域的NF186组分进行蛋白质印迹。从PubMed检索CIDP并发FSGS的病例并进行审查。
结果：我们报道了一名66岁的中国女性，患有CIDP样自身免疫性脑神经病并并发FSGS。她的NF186抗体阳性。在患有微小肾病的患者的肾组织切片中检测到NF186的荧光信号。NF186的染色在空间上与足细胞匹配。在西方印迹分析中，患者的血清中有识别NF186蛋白的抗体,并且他们的抗体识别NF186的Ig结构域.已报道3例NF186抗体和FSGS阳性的CIDP样自身免疫性神经病变。所有这些患者对皮质类固醇有反应，而不是静脉注射免疫球蛋白，就神经病变和肾脏疾病而言。
结论：NF186可能是并发FSGSinCIDP-样自身免疫性性神经病的发病机制中的靶抗原，NF186抗体阳性。NF186抗体和FSGS阳性的CIDP样自身免疫性神经病是一种罕见的实体，可能对皮质类固醇联合免疫抑制剂有反应。
方法：我们介绍了一例伴有FSGS的CI-DP样自身免疫性神经病变。我们通过细胞结合测定(CBA)方法测量NF186抗体。我们使用兔抗NF186抗体或NF186抗体阳性人血清对患有轻度肾病的患者的肾冷冻切片样品进行了免疫荧光分析。然后我们对与人血清和相应的兔多克隆抗体一起孵育的重组NF186蛋白和包括Ig和FNIII结构域的NF186组分进行蛋白质印迹。从PubMed检索CIDP并发FSGS的病例并进行审查。
结果：我们报道了一名66岁的中国女性，患有CIDP样自身免疫性脑神经病并并发FSGS。她的NF186抗体阳性。在患有微小肾病的患者的肾组织切片中检测到NF186的荧光信号。NF186的染色在空间上与足细胞匹配。在西方印迹分析中，患者的血清中有识别NF186蛋白的抗体,并且他们的抗体识别NF186的Ig结构域.已报道3例NF186抗体和FSGS阳性的CIDP样自身免疫性神经病变。所有这些患者对皮质类固醇有反应，而不是静脉注射免疫球蛋白，就神经病变和肾脏疾病而言。
结论：NF186可能是并发FSGSinCIDP-样自身免疫性性神经病的发病机制中的靶抗原，NF186抗体阳性。NF186抗体和FSGS阳性的CIDP样自身免疫性神经病是一种罕见的实体，可能对皮质类固醇联合免疫抑制剂有反应。
