Abstract
Neuromyelitis optica spectrum disorders (NMOSDs) are inflammatory diseases with a high risk of recurrence and progressive disability, and it is crucial to find sensitive and reliable biomarkers for prognosis and the early prediction of relapse. Highly active NMOSD is defined as two or more clinical relapses within a 12-month period. In this study, we analyzed independent risk factors among patients with aquaporin-4 (AQP4)-IgG positive highly active NMOSD. In this retrospective study, we analyzed the data of 94 AQP4-IgG positive patients with highly active NMOSD and 105 AQP4-IgG positive controls with non-highly active NMOSD. In order to rule out possible effects of previous treatments (such as glucocorticoids, immunoglobulin, and immunosuppressants), we focused on the first-attack NMOSD patients admitted to our hospital. Clinical data, including the age of onset, gender, comorbidities, and serum analysis and cerebrospinal fluid (CSF) analysis results, were collected, after which logistic regression models were used to determine the associations between the clinical factors and relapse outcomes. The prevalence of connective tissue disease and the proportion of antinuclear antibody (ANA)-positivity were higher in the highly active NMOSD group than in the control group. The leukocyte counts, homocysteine (Hcy) levels, CSF leukocyte counts, protein concentrations, IgG indexes, and 24h IgG synthesis rates were also higher in the highly active NMOSD group. The results of multivariate analysis indicated that connective tissue disease comorbidity (OR = 5.953, 95% CI: 1.221–29.034, P = 0.027), Hcy levels (OR = 1.063, 95% CI: 1.003–1.126, P = 0.04), and 24h IgG synthesis rate (OR = 1.038, 95% CI: 1.003–1.075, P = 0.034) may be independent risk factors for AQP4-IgG positive highly active NMOSD relapse after adjusting for various variables. Comorbidity of connective tissue disease, Hcy levels, and 24h IgG synthesis rate may be independent risk factors for AQP4-IgG positive highly active NMOSD.
Highlights
Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune diseases characterized by unpredictable attacks on the optic nerves, spinal cord, brainstem, and other areas of the central nervous system, which result in an accumulation of neurological disability
We focused on the first-attack NMOSD patients admitted to our hospital in order to rule out possible effects of previous treatments and accurately calculate the times of relapse events during the follow-up
Of the 199 cases of AQP4-IgG positive first-attack NMOSD who were admitted to our hospital, 94 met the inclusion criteria for highly active NMOSD in accordance with the specified relapse times during the 12 month-follow-up after the first attack, and 105 AQP4IgG positive cases were enrolled as controls
Summary
Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune diseases characterized by unpredictable attacks on the optic nerves, spinal cord, brainstem, and other areas of the central nervous system, which result in an accumulation of neurological disability. NMOSD has a wide spectrum of clinical features, including optic neuritis, longitudinally extensive transverse myelitis, diencephalic syndrome, and other encephalitic presentations. Patients with NMOSD have a high risk of recurrence and a high incidence of disability. The prognosis of relapsing NMOSD is usually poor, among patients with frequent relapses [1,2,3]. Researchers have attempted to find reliable and sensitive markers to predict NMOSD relapses and prognosis. Active NMOSD, known as highly relapsing NMOSD, is defined as at least two clinical relapses during the previous 12 months. Previous studies showed that the comorbidity burden was significantly higher among patients with highly active NMOSD compared with the overall NMOSD population [4, 5]. The association between comorbidity and relapse has not been further analyzed
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