Anti-NF155/NF186/CNTN1/Caspr1 IgG Antibodies And Study Of Related Clinical Features In Patients With Chronic Inflammatory Demyelinating Polyneuropathy

Objective: Chronic inflammatory demyelinating polyneuropathy(CIDP)is a spectrum of autoimmune-mediated chronic peripheral neuropathy with significant clinical heterogeneity,which is characterized by progressively symmetrical distal or proximal paralysis and immunotherapy effectiveness.Recent studies showed that antiNF155/NF186/CNTN1/Caspr1 IgG antibodies specifically bind to peripheral nerve node/paranode of Ranvier,causing node/paranode dysfunction;Specific antibody-positive CIDP patients present heterogeneity that include clinical manifestations and response to immune treatments.In this study,we detected the expression of antiNF155/NF186/CNTN1/Caspr1 IgG antibodies in the serum of CIDP patients from different provinces in China,compared antibody positive with negative patients' clinical features,and explored clinical features of antibody-positive CIDP patients compared with other countries studies.Methods: Collected the clinical data and peripheral venous blood of 29 CIDP patients(22 were from Shandong province,5 were from Hunan province,1 was from Jiangsu province and 1 was from Anhui province).Detected anti-NF155/NF186/CNTN1/Caspr1 antibodies in patients by cell-based binding assay and teased mouse sciatic nerve fibers binding assay.Compared the clinical features of specific antibody-positive patients with antibody-negative by t-test or Fisher's exact test;And analyzed the heterogeneity of antibody-positive CIDP patients in different countries by chi-square.Normally distributed continuous data were presented as?? ± S,and categorical variables were shown as %(n/N).Analysis was performed with the SPSS 20.0 analysis software.A two-sided P value < 0.05 was considered to be significant.Results: In this study,the median age of onset age of 29 patients with CIDP was 41 years old(INR,33-61);the ratio of male to female was 1.4;3.8% of patients complained of antecedent events;and the main clinical symptom was limb weakness(62.1%).89.3% of 28 patients treated with glucocorticoids and 63.3% of 11 patients treated with intravenous immunoglobulin(IVIg)were responsive to the treatment.In this study,the positive rate of anti-NF155 IgG antibodies in this study was 20.4%,which is similar to Japanese CIDP patients(18%)and higher than American CIDP patients(10%).In this study,1 patient(3.4%)was positive for antiNF186 IgG antibody;Nobody was positive for anti-CNTN1/Caspr1 IgG antibodies.In this study,compared with antibody-negative patients,the onset age of anti-NF155 IgG antibody-positive patients was younger(33.8 ± 5 years old vs 48 ± 16 years old,P < 0.05);the clinical features of antiNF155 IgG antibody-positive patients were mainly distal limb weakness,and showed a poor response to IVIg treatment;Those results in this study were not different from Japanese and American CIDP patients.Onset of the anti-NF155 IgG antibody-positive CIDP in this study was mainly chronic,which is similar to Japanese CIDP patients and higher than American CIDP patients(P < 0.05).Comparing with Japanese CIDP patients,the clinical types of anti-NF155 IgG antibody-positive CIDP patients were predominant pure motor(66.7% vs 0,P < 0.05).Comparing limb weakness,paresthesia,ataxia or cranial nerves involvement conditions of anti-NF155 IgG antibody-positive CIDP patients in different countries,there were no statistical difference(P > 0.05).53.8% of antiNF155 IgG antibody-positive Japanese CIDP patients were accompanied with tremor,which is higher than CIDP patients in this study(16.7%)and American(0).CSF protein elevated levels of anti-NF155 IgG antibodypositive patients in our study were lower than Japanese and American CIDP patients(0.8 ± 0.5g/L vs 3.17 ± 1.4g/L,P < 0.05;0.8 ± 0.5g/L vs 1.5 ± 0.4g/L,P < 0.05).Comparing glucocorticoid and IVIg treatment responsive of anti-NF155 IgG antibody-positive CIDP patients in different countries,there were no statistical difference.The clinical features of a patient with anti-NF186 IgG antibody-positive CIDP in this study were chronic onset,severe lower limbs paralysis,and responsive for glucocorticoid and IVIg treatment.Conclusion: In this study,anti-NF155 IgG antibody-positive patients showed younger onset age and resisted to IVIG treatment,which were similar to Japanese and American CIDP patitents.Compared with antiNF155 IgG antibody-positive CIDP patients in Japan and American,CIDP patients in this study were similar in onset age,but there were differences in onset methods,clinical classification,and accompanying symptoms.The results of our study need to be confirmed by large sample and multicenter studies.