The Clinical Characteristics And Prognosis Of Neuromyelitis Optica Spectrum Disorders With Autoimmune Diseases

ObjectiveExplore the clinical characteristics and prognosis of neuromyelitis optica spectrum disorders with autoimmune diseases,and provide evidence for early diagnosis and treatment of this kind of diseases.Materials and meyhodsContinuous collection of 101 NMOSD patients from the inpatient and outpatient departments of the First Affiliated Hospital of Fujian Medical University from January 2015 to October 2019,including 20 cases of Sj?gren’s syndrome and 7 cases of systemic lupus erythematosus,3 cases of rheumatoid arthritis,12 cases of autoimmune t-hyroiditis,1 case of myasthenia gravis,and the rest 58 cases of simple NMOSD.Collect each patient’s gender,age of onset,EDSS score before admission,MRI lesion involvement distribution,AQP4 antibody titer,serum and cerebrospinal fluid laboratory related indicators,before using sequential treatment regimen in remission and at least3 treatments EDSS scores after months,where the difference in rates is taken by the chisquare test or Fisher’s exact probability method,the difference between groups is by the independent sample t test or Mann-Whitney U test,and the Pearson correlation is used to test the correlation between the two variables.Patients with other autoimmune diseases and patients with simple NMOSD have different clinical effects,laboeatory tests,imaging tests,and different sequential treatments in remission.Results1.Compared with the simple group,the patients in the SS group had a higher EDSS score before treatment(4.83 ± 1.38 vs.3.48 ± 1.22,P <0.001),and were more likely to relapse within 1 year(50% vs.24.1%,P = 0.034),Cervical thoracic spinal cord involvement is higher(45% vs.17.2%,P = 0.028).In laboratory tests,serum chloride and cerebrospinal fluid chloride in the SS group are slightly higher than those in the simple group [109(104,112)vs.104(101,106.5),P = 0.002;122.7 ± 3.08 vs.119.79 ± 3.30,P = 0.026],the number of white blood cells and protein content of cere-brospinal fluid were higher than that of the simple group [20(12,43)vs.8(5,12),P =0.002;0.45(0.39,0.48)vs.0.27(0.2,0.33),P <0.05].The difference between the comb-ined group and the simple group in the level of AQP4 antibody titer has no statistical significance(P> 0.05).In sequential treatment with azathioprine during remission,the EDSS score of the combined SS group before treatment was significantly higher than that of the simple group(4.59 ± 1.00 vs.3.65 ± 1.18,P = 0.026),The EDSS scores of the two groups after treatment were lower than before treatment,and the difference was statistically significant [4.59 ± 1.00 vs.3.5(3,4),P = 0.003;3.65 ± 1.18 vs.3(2.5,3.5);P < 0.001],and there was no difference in EDSS scores after treatment(P> 0.05).After using low-dose hormone therapy,the EDSS scores of the two groups were lower than before treatment,the difference was statistically significant [5.08 ± 1.72 vs.4.33 ± 1.63,P = 0.030;3.16 ± 1.25 vs.2.87 ± 1.16,P = 0.044].2.Compared with the NMOSD group,there was no statistically significant difference in gender and first age in the SLE group(P> 0.05),the proportion of cervical and thoracic spinal cord damage was high(100% vs.17.2%,P <0.001),serological antinuclear antibodies,anti-SSA antibody,anti-SSB antibody and anti-Ro-52 antibody positive rate was higher(P<0.05);cerebrospinal fluid pressure,cerebrospinal fluid lactic acid,sugar content,cerebrospinal fluid white blood cell count,protein content,chloride,AQP4 antibody drops between the two groups,there was no statistically significant difference in degree(P> 0.05);there were no statistically significant differences in EDSS scores before and after treatment with methotrexate,cyclophosphamide,and azathioprine in 7 patients with SLE(P> 0.05).3.Compared with the simple group,there was no statistically significant difference in gender and first age in the combined AITD group(P> 0.05),the proportion of intracranial lesions involving the medulla oblongata was high(66.7% vs.25.9%;P =0.016),CSF protein quantification,thyroid The incidence of T3 is lower with low function [0.53 ± 0.23 vs.0.27 ± 0.09,P = 0.002;66.7% vs.11.8%,P=0.009].However,under azathioprine treatment,there was no significant difference in the EDSS scores between the combined group and the simple group before and after treatment(P>0.005),and the EDSS scores of the two groups after treatment were lower than before treatment [4.07 ± 1.10 vs.3(2.5,4),P = 0.039;3.65±1.18 vs.3(2.5,3.5),P <0.001].Conclusion:1.Compared with patients with NMOSD,patients with SS and SLE are more likely to have spinal cord injury distributed in the cervical thoracic spinal cord.Patients with SS have a higher EDSS score before treatment,a higher relapse rate within 1 year,and a higher positive rate of other autoimmune antibodies.The quantitative analysis of cerebrospinal fluid white blood cell count,trace protein and sugar has certain reference value in the diagnosis and treatment of NMOSD supporting diagnosis with SS,and it should be observed in the clinic.2.The use of azathioprine in the remission phase of NMOSD patients with SS has a greater benefit than that of patients with simple NMOSD,so special attention should be paid to the long-term monitoring and follow-up of this population,and azathioprine can be considered as routine treatment for patients with SS drug.3.Compared with the simple group,patients with combined AITD have a higher proportion of medullary lesions and often have low T3.Most T3 levels are negatively correlated with EDSS scores.T3 levels may become in dicators for predicting patient prognosis.Pay attention to the improvement and dynamic monitoring of this indicator.