Clinical Prediction Model Of Relapse After The First Episode Of Neuromyelitis Optic Spectrum Disease

Background and Objective:Neuromyelitis optica spectrum disorders(NMOSD)is an immune-mediated inflammatory demyelinating disease of the central nervous system,the main clinical features are recurrent optic neuritis(ON)and longitudinally extensive transversemyelitis(LETM),which can lead to severe disability.The prevalence of NMOSD ranged from 0.52 to 4.4 per 100,000 population.This disease is more common in Asian patients,the average age of onset is 39 years old,male-female ratio is 1:5-10.NMOSD is a highly recurrent and highly disabling disease.More than 90%of patients have multiple stages of disease,and about 60%of patients relapse within 1 year.Most patients are left with severe visual impairment or limb dysfunction,urinary and fecal dysfunction.The study found that the factors affecting the recurrence of NMOSD patients may be related to race,gender,age,co-underlying autoimmune diseases,history of prodromal infection,serum aquaporin-4-IgG(AQP4-IgG)results,core clinical characteristics of the first episode and the use of immunosuppressive agents.However,there is still a lack of clinical prediction model for recurrence in NMOSD patients.This study aims to construct a clinical prediction model for recurrence in NMOSD patients and to explore its predictive value by screening the influencing factors of recurrence in NMOSD patients.Methods:We developed a clinical prediction model based on a training dataset of 278 NMOSD patients,and data were collected from July 2015 to January 2019,in The First Affiliated Hospital of Zhengzhou University.Multivariate logistic regression analysis method is adopted to establish the prediction model.To assess the identification,calibration,and clinical utility of the predictive model using the c index,calibration map,and decision curve analysis.Internal validation was assessed using the bootstrapping validation.Results:Predictors contained in the prediction nomogram included age,sex,prodromal infection,first clinical symptoms,immunosuppressive use,whether to combine with other autoimmune diseases and serum AQP4-IgG results.The model displayed good discrimination with a C-index of 0.761(95%confidence interval:0.70514-0.81686)and good calibration.High C-index value of 0.74 could still be be verified in the interval validation.Decision curve analysis showed that the recurrence within one year nomogram was clinically useful when intervention was decided at the recurrence within one year possibility threshold of 16%-84%.Conclusions:1.Patients with NMOSD who were precursors to infection,first onset of brain/brainstem syndrome,not taking immunosuppressant regularly in remission,combined with other autoimmune diseases were more likely to relapse within 1 year of the first attack,with statistically significant differences(p<0.05).2.There was no significant statistical difference in gender,age,first symptom of optic neuritis or acute myelitis,serum AQP4-IgG result between the recurrence group and the non-recurrence group within 1 year after the first attack(p>0.05).3.This study developed a new clinical predictive model with good accuracy to help clinicians understand the risk of recurrence within one year of NMOSD.