| ObjectiveNeuromyelitis optica spectrum disorder(NMOSD)is an autoimmune inflammatory demyelinating disease that often occurs in the optic nerve and spinal cord.Since the detection of AQP4 antibody as a specific antibody,the number of NMOSD patients has increased significantly,and more and more patients have been found to be accompanied by Connective tissue disease(CTD),but most of the related reports are case reports.There are few studies on the clinical,laboratory,imaging and prognosis of patients with NMOSD complicated with CTD.This study will analyze and explore the demographic characteristics,clinical manifestations,serum immunological tests,imaging features and prognostic characteristics of NMOSD patients with CTD,and compare them with simple NMOSD patients,in order to improve the understanding of neurologists,rheumatologist and ophthalmologists about this kind of disease,avoid missed diagnosis and misdiagnosis,and reduce their recurrence and disability,and improve the quality of life of patients.Methods1.32 patients with AQP4 antibody positive NMOSD diagnosed in the People’s Hospital of Zhengzhou University from December 2017 to June 2019 and combined with CTD and 64 patients with pure AQP4 antibody positive NMOSD diagnosed in the same period were collected as the study subjects.2.The clinical data of the patients were collected,including sex,age of onset,first symptoms,main clinical manifestations,serological immune index test results,imaging findings,EDSS score,teeatment methods,annual recurrence rate and other related data,and the two groups were analyzed.3.Statistical analysis using SPSS 24.0 software,t-test,chi-square test and rank sum test were used to analyze the data of the two groups,and Spearman analysis was used to analyze the correlation.The difference was statistically significant when P<0.05.ResultsAccording to the diagnostic criteria of NMOSD in 2015,96 patients were included,and all of them were positive for AQP4 antibody,including 79 females(82.3%)and 17 males(17.7%).The ratio of female to male was 4.6:1.The patients were divided into two groups:simple NMOSD group and NMOSD group with CTD.There were 64 patients in the simple NMOSD group,including 15 males(23.4%)and 49 females(76.6%).The age of onset was 8 to 75 years old,the average age was 39.98 ± 16.694 years old.There were 32 patients with CTD,including 28 patients with Sjogren’s syndrome((SS)),3 patients with systemic lupus erythematosus((SLE)),1 patient with both diseases,and 2 patients with rheumatoid arthritis((RA)).There are 2 males(6.25%)and 30 females(93.75%).The age of onset was 6 to 68 years old,the average age was 38.34 ± 15.553 years old.Compared with the simple NMOSD group,the sex ratio of the patients with CTD was significantly different,and the female patients were more likely to develop NMOSD with CTD(P=0.038).There was no significant difference in the age of onset between the two groups.The first symptoms of the two groups were vision loss,sensory disturbance,limb weakness,nausea and vomiting,and there was no significant difference between the two groups.The main clinical manifestations in the course of the disease were retention of urine and stool,diplopia,change of consciousness,dysphagia and dysarthria.There was no significant difference in symptoms between the two groups.MRI examination of head and spinal cord was performed in all patients.Optic nerve involvement was the most common in the two groups,and there was no significant difference in the distribution of optic nerve and intracranial lesions between the two groups(P>0.05).There were 36cases of spinal cord involvement in patients with simple NMOSD and 19 cases in patients with CTD,all of which were the most common in cervical spinal cord involvement,but there was no significant difference in the distribution of lesions on the spinal cord between the two groups(P>0.05).The long segmental lesions of spinal cord lesions with lesion length>3 in patients with simple NMOSD accounted for 28.1%,and 50%of NMOSD patients with combined CTD,P=0.035<0.05,which was statistically significant.In terms of serological immune indexes,there was no significant difference in AQP4 antibody titer between patients with simple NMOSD and patients with NMOSD with CTD.Autoantibodies ANA,anti-SSA antibody,anti-SSB antibody and Ro-52 antibody were expressed in both groups,but the level of autoimmune antibody in patients with NMOSD complicated with CTD was significantly higher than that in patients with simple NMOSD.The treatment plans of the two groups were basically the same.Glucocorticoid/gamma globulin was given alone or in combination in the acute phase of the disease.In the remission stage,some patients were treated with long-term immunosu-ppressants to prevent recurrence.45 patients(70.3%)had simple NMOSD recurrence,and the annual recurrence rate was 1.00(0.45,1.00),NMOSD with CTD.The annual recurrence rate of NMOSD patients with CTD was 1.00(0.535,3.00),P=0.043<0.05,Patients with NMOSD combined with CTD were more likely to relapse,and the difference was statistically significant.After receiving immunosuppressant treatment,there was no significant difference in the annual recurrence rate between the two groups(P>0.05).The EDSS score of the patients with simple NMOSD was 1.25(1,4),and that of the patients with CTD was 2(1,3.875).There was no significant difference in the EDSS score between the two groups.The results of Spearman correlation analysis showed that EDSS score was not correlated with grouping(simple NMOSD and NMOSD with CTD),and annual recurrence rate,but positively correlated with long segmental myelitis with continuous lesion length of spinal cord>3 vertebae(ritual=0.281,P=0.015).Conclusion1.Compared with simple NMOSD patients,NMOSD patients with CTD were more likely to develop the disease in women,and the recurrence rate was higher;2.The positive rate of ANA,anti-SSA,anti-SSB and Ro-52 antibodies in serum of NMOSD patients with CTD was higher,and long segmental myelitis with continuous focus length>3 vertebrae was more likely to occur. |
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