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      Neuromielitis óptica y lupus eritematoso sistémico: caso clínico Translated title: Neuromyelitis optica and systemic lupus erythematosus: clinical case


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          Resumen La neuromielitis óptica (NMO) es una enfermedad desmielinizante del sistema Nervioso Central, con morbilidad, mortalidad alta, con respuesta favorable al tratamiento inmunosupresor y asociación infrecuente a enfermedades inmunológicas como Lupus Eritematoso Sistémico. Se reporta caso de un adolescente con diagnóstico de Neuromielitis óptica que presento durante su evolución Lupus Eritematoso Sistémico. Su evolución fue adecuada por la respuesta favorable a terapia inmunosupresora.

          Translated abstract

          Abstract Optic neuromyelitis (NMO) is a demyelinating disease of the Central Nervous System, with morbidity, high mortality, favorable response to immunosuppressive treatment and infrequent association to immunological diseases such as Systemic Lupus Erythematosus. We report the case of a teenager with a diagnosis of Optic Neuromyelitis who presented Systemic Lupus Erythematosus during his evolution. Its evolution was adequate due to the favorable response to immunosuppressive therapy.

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          Most cited references19

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          The spectrum of neuromyelitis optica.

          Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.
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            Antibody to aquaporin-4 in the long-term course of neuromyelitis optica

            Neuromyelitis optica (NMO) is a severe inflammatory CNS disorder of putative autoimmune aetiology, which predominantly affects the spinal cord and optic nerves. Recently, a highly specific serum reactivity to CNS microvessels, subpia and Virchow–Robin spaces was described in patients with NMO [called NMO–IgG (NMO–immunoglobulin G)]. Subsequently, aquaporin-4 (AQP4), the most abundant water channel in the CNS, was identified as its target antigen. Strong support for a pathogenic role of the antibody would come from studies demonstrating a correlation between AQP4-Ab (AQP4-antibody) titres and the clinical course of disease. In this study, we determined AQP4-Ab serum levels in 96 samples from eight NMO–IgG positive patients (median follow-up 62 months) in a newly developed fluorescence-based immunoprecipitation assay employing recombinant human AQP4. We found that AQP4-Ab serum levels correlate with clinical disease activity, with relapses being preceded by an up to 3-fold increase in AQP4-Ab titres, which was not paralleled by a rise in other serum autoantibodies in one patient. Moreover, AQP4-Ab titres were found to correlate with CD19 cell counts during therapy with rituximab. Treatment with immunosuppressants such as rituximab, azathioprine and cyclophosphamide resulted in a marked reduction in antibody levels and relapse rates. Our results demonstrate a strong relationship between AQP4-Abs and clinical state, and support the hypothesis that these antibodies are involved in the pathogenesis of NMO.
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              Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses.

              Neuromyelitis optica (NMO) attacks often are severe, are difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks.

                Author and article information

                Gaceta Médica Boliviana
                Gac Med Bol
                Facultad de Medicina de la Universidad Mayor de San Simón (Cochabamba, , Bolivia )
                : 46
                : 1
                : 117-120
                [1] orgnameTrasplante Renal Pediátrico
                [2] orgnameDermatología Pediátrica
                [3] Cochabamba Cochabamba orgnameUniversidad Mayor de San Simón orgdiv1Facultad de Medicina Bolivia
                S1012-29662023000100117 S1012-2966(23)04600100117

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                : 12 January 2023
                : 20 March 2023
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 19, Pages: 4

                SciELO Bolivia

                CASOS CLÍNICOS

                neuritis óptica,optic neuritis,devic neuromielytis optica,systemic Lupus erythematosus,neuromielitis óptica óptica de Devic,lupus eritematoso sistémico


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