Shufan Zhang1, Shilin Yang1, Jiahong Lu1, Shaojun Liu2, Weicheng Wu3, Mingshi Gao4, Jie Lin1, Xiangjun Chen1, Dongqing Zhu1, Shuang Ye5, Chun Yu1, Shufen Chen1, Qiang Dong1, Bing Zhu6, Xiang Han7. 1. Department of Neurology, Huashan Hospital, Fudan University, No. 12, Wulumuqi Road, Jing'an District, Shanghai, 200040, China. 2. Department of Renal Pathology, Huashan Hospital, Fudan University, No. 796, Jiangsu Road, Jing'an District, Shanghai, 200040, China. 3. School of Life Sciences, Fudan University, No. 2205, Songhu Road, Yangpu District, Shanghai, 200438, China. 4. Department of Pathology, Huashan Hospital, Fudan University, No. 12, Wulumuqi Road, Jing'an District, Shanghai, 200040, China. 5. Department of Rheumatology and Immunology, Renji Hospital, Shanghai Jiaotong University, No. 160, Pujian Road, Pudong New District, Shanghai, 200127, China. 6. Kindstar Global Precision Medicine Institute, No. 666 Gaoxin Dadao, Donghu New Technology Development District, Wuhan, 430000, China. 7. Department of Neurology, Huashan Hospital, Fudan University, No. 12, Wulumuqi Road, Jing'an District, Shanghai, 200040, China. hansletter@fudan.edu.cn.
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.
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.