TY - JOUR AU1 - Zhang, Shufan AU2 - Yang, Shilin AU3 - Lu, Jiahong AU4 - Liu, Shaojun AU5 - Wu, Weicheng AU6 - Gao, Mingshi AU7 - Lin, Jie AU8 - Chen, Xiangjun AU9 - Zhu, Dongqing AU1 - Ye, Shuang AU1 - Yu, Chun AU1 - Chen, Shufen AU1 - Dong, Qiang AU1 - Zhu, Bing AU1 - Han, Xiang AB - BackgroundThis 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.MethodsWe 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.ResultsWe 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.ConclusionsNF186 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. TI - CIDP-like autoimmune nodopathy complicated with focal segmental glomerulosclerosis: a case study and literature review JF - Journal of Neurology DO - 10.1007/s00415-022-11369-4 DA - 2023-01-01 UR - https://www.deepdyve.com/lp/springer-journals/cidp-like-autoimmune-nodopathy-complicated-with-focal-segmental-FX9eGX4KeK SP - 493 EP - 502 VL - 270 IS - 1 DP - DeepDyve ER -