Abstract
BackgroundNeuromyelitis optica spectrum disorders (NMOSDs) are attack-relapsing autoimmune inflammatory diseases of the central nervous system, which are characterized by the presence of serological aquaporin-4 (AQP4) antibody. However, this disorder is uncommon in children, and AQP4 antibody was often found to be seronegative. However, some pediatric patients diagnosed with NMOSDs were tested to be positive for myelin oligodendrocyte glycoprotein (MOG) antibody. The previous investigations of pediatric NMOSDs were usually focused on the clinical presentation, treatment responses, and long-term prognoses, but little is known about the risk factors predicting NMOSD relapse attacks in a shorter time, especially, for Chinese children.MethodsWe retrospectively identified 64 Chinese pediatric patients, including 39 positive for AQP4 antibody, 12 positive for MOG antibody, and the rest negative for AQP4 and MOG antibodies. Independent risk factors predicting relapse in 1-year follow-up were extracted by multivariate regression analysis to establish a risk score model, its performance evaluation was analyzed using receiver operating characteristic (ROC) curve, and the independent risk factors related to relapse manifestation were also explored through multivariate logistic analysis. A nomogram was generated to assess relapse attacks in 1-year follow-up. Thirty-five patients from 3 other centers formed an external cohort to validate this nomogram.ResultsFour independent relapsed factors included discharge Expanded Disability Status Scale (EDSS) (p = 0.017), mixed-lesion onset (p = 0.010), counts (≧1) of concomitant autoantibodies (p = 0.015), and maintenance therapy (tapering steroid with mycophenolate mofetil (MMF), p = 0.009; tapering steroid with acetazolamide (AZA), p = 0.045; and tapering steroid only, p = 0.025). The risk score modeled with these four factors was correlated with the likelihood of relapse in the primary cohort (AUC of 0.912) and the validation cohort (AUC of 0.846). Also, our nomogram exhibited accurate relapse estimate in the primary cohort, the validation cohort, and the whole cohort, but also in the cohorts with positive/negative AQP4 antibody, and noticeably, it performed predictive risk improvement better than other factors in the concordance index (C-index), net reclassification improvement (NRI), and integrated discrimination improvement (IDI).ConclusionsThe risk score and nomogram could facilitate accurate prognosis of relapse risk in 1-year follow-up for pediatric NMOSDs and help clinicians provide personalized treatment to decrease the chance of relapse.
Highlights
Neuromyelitis optica spectrum disorders (NMOSDs) are defined as a category of autoantibody-induced central nervous system (CNS) inflammatory diseases characterized by recurrent attacks targeting the optic nerves, spinal cord, or brain/brainstem
Recent studies revealed that some children with NMOSD could acquire AQP4-negative autoimmunity [7, 8], indicating that myelin oligodendrocyte glycoprotein (MOG) antibody targeting MOG antigen on myelin sheaths of oligodendrocyte [9], mainly concomitant with CD4+ T cells and granulocyte infiltration, and complement deposition not observed on astrocytes or glia limitans [10], was detectable in some pediatric patients with AQP4 antibodynegative NMOSDs [11]
Some pediatric patients with MOG antibody-positive NMOSDs have clinical overlapped manifestations with AQP4 antibody-positive NMOSDs [23], despite the distinct pathology evidence characteristic of severe demyelination without the astrocyte loss usually recognized in AQP4 antibody-positive NMOSD [24]
Summary
Neuromyelitis optica spectrum disorders (NMOSDs) are defined as a category of autoantibody-induced central nervous system (CNS) inflammatory diseases characterized by recurrent attacks targeting the optic nerves, spinal cord, or brain/brainstem. As for Chinese children, NMOSDs are considered as the common type of acquired CNS demyelinating diseases [6]; it remains elusive regarding the relapse risk factors and even the clinical course and prognosis for Chinese pediatric NMOSDs. Neuromyelitis optica spectrum disorders (NMOSDs) are attack-relapsing autoimmune inflammatory diseases of the central nervous system, which are characterized by the presence of serological aquaporin-4 (AQP4) antibody. Neuromyelitis optica spectrum disorders (NMOSDs) are attack-relapsing autoimmune inflammatory diseases of the central nervous system, which are characterized by the presence of serological aquaporin-4 (AQP4) antibody This disorder is uncommon in children, and AQP4 antibody was often found to be seronegative. The previous investigations of pediatric NMOSDs were usually focused on the clinical presentation, treatment responses, and long-term prognoses, but little is known about the risk factors predicting NMOSD relapse attacks in a shorter time, especially, for Chinese children
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