Contemporary management challenges in seropositive NMOSD

Costello, Fiona; Burton, Jodie M.

doi:10.1007/s00415-022-11241-5

Cited by 10 publications

(11 citation statements)

References 72 publications

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Section: A New Treatment Eramentioning

confidence: 99%

“…Autologous hematopoietic stem cell bone marrow transplantation is an immune reconstitution therapy that has also been used successfully to treat patients with NMOSD 94-98 . Small studies using transplantation techniques have shown clinical benefit, radiologic remission, improved disability, and suppression of AQP4-IgG antibody production 94-98 . The European Group for Blood and Marrow Transplantation Autoimmune Diseases Working Party 95 reported that 3 of 16 NMOSD cases demonstrated resolution of disease progression and required no additional immunosuppressive treatment after a median follow-up period of 47 months.…”

Section: A New Treatment Eramentioning

confidence: 99%

“…The evidence to date suggests that autologous hematopoietic stem cell transplantation may be used as salvage therapy for severe breakthrough disease to reconstitute the immune system. This treatment may also serve as an induction therapy for severe initial presentations of NMOSD before the initiation of long-term immunomodulation 94-99 . In the short term, immune reconstitution therapies have advantages over immunosuppressive agents by providing long-term efficacy with minimal risk for opportunistic infections and malignancy 94 .…”

Section: A New Treatment Eramentioning

confidence: 99%

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Neuromyelitis Optica Spectrum Disorders

Costello¹

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW: This article reviews the cardinal clinical features, distinct immunopathology, current diagnostic criteria, relapse-related risk factors, emerging biomarkers, and evolving treatment strategies pertaining to neuromyelitis optica spectrum disorders (NMOSD).RECENT FINDINGS: The discovery of the pathogenic aquaporin-4 (AQP4)-IgG autoantibody and characterization of NMOSD as an autoimmune astrocytopathy have spearheaded the identification of key immunologic therapeutic targets in this disease, including but not limited to the complement system, the interleukin 6 (IL-6) receptor, and B cells. Accordingly, four recent randomized controlled trials have demonstrated the efficacy of three new NMOSD therapies, namely eculizumab, satralizumab, and inebilizumab.SUMMARY: Currently, NMOSD poses both diagnostic and treatment challenges. It is debated whether individuals who are seropositive for myelin oligodendrocyte glycoprotein (MOG)-IgG belong within the neuromyelitis optica spectrum. This discussion is fueled by disparities in treatment responses between patients who are AQP4-IgG seropositive and seronegative, suggesting different immunopathologic mechanisms may govern these conditions. As our understanding regarding the immune pathophysiology of NMOSD expands, emerging biomarkers, including serum neurofilament light chain and glial fibrillary acidic protein (GFAP), may facilitate earlier relapse detection and inform long-term treatment decisions. Future research focal points should include strategies to optimize relapse management, restorative treatments that augment neurologic recovery, and practical solutions that promote equitable access to approved therapies for all patients with NMOSD.

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Section: A New Treatment Eramentioning

confidence: 99%

Section: A New Treatment Eramentioning

confidence: 99%

Section: A New Treatment Eramentioning

confidence: 99%

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Neuromyelitis Optica Spectrum Disorders

Costello¹

2022

CONTINUUM: Lifelong Learning in Neurology

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“…Relapse events are typically based on subjective clinical judgement, the emergence of new or worsening symptoms consistent with NMOSD, and/or evidence of lesions on imaging. Consistent with established clinical practices, the defined duration for all relapses was set to 90 days [34][35][36][37][38][39]. Therefore, to harmonize relapse history and minimize the impact of recall bias or variability, relapses that began within 90 days before study consent were counted as on-study relapses, and those starting > 90 days prior to consent were considered pre-study relapses.…”

Section: Discussionmentioning

confidence: 99%

Clinical and epidemiological correlates of treatment change in patients with NMOSD: insights from the CIRCLES cohort

Gholizadeh¹,

Exuzides²,

Lewis

et al. 2022

J Neurol

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Objective Neuromyelitis optica spectrum disorders (NMOSD) represent rare autoimmune diseases of the central nervous system largely targeting optic nerve(s) and spinal cord. The present analysis used real-world data to identify clinical and epidemiological correlates of treatment change in patients with NMOSD. Methods CIRCLES is a longitudinal, observational study of NMOSD conducted at 15 centers across North America. Patients with ≥ 60 days of follow-up and receiving on-study maintenance treatment were evaluated. The mean annual relapse rate (ARR) was estimated using negative binomial models; the likelihood of treatment change was estimated using Cox proportional hazards models. Relapses were included as time-varying covariates to estimate the relationship to treatment change. Results Of 542 patients included, 171 (31.5%) experienced ≥ 1 relapse on the study and 133 patients (24.5%) had ≥ 1 change in the treatment regimen. Two categories of variables significantly correlated with the likelihood of treatment change: (1) relapse: any on-study relapse (hazard ratio [HR] = 2.91; p < 0.001), relapse phenotypes (HR range = 2.15–5.49; p < 0.001), and pre-study ARR > 0.75 (HR 2.28; p < 0.001); 2) disease phenotype: brain syndrome only vs transverse myelitis involvement at onset (HR 2.44; p = 0.008), disease duration < 1 vs > 5 years (HR 1.66; p = 0.028), or autoimmune comorbidity (HR 1.55; p = 0.015). A subset of these factors significantly correlated with shorter time to first rituximab discontinuation. Conclusions In CIRCLES, relapse patterns and disease phenotype significantly correlated with changes in the maintenance treatment regimen. Such findings may facilitate the identification of patients with NMOSD who are likely to benefit from treatment change to reduce relapse risk or disease burden and enhance the quality of life.

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“…The 2015 revised international consensus criteria base the diagnosis of NMOSD on the presence of core clinical characteristics and serum AQP‐4 autoantibodies 11,13–15 . Besides, other alternative diagnoses such as multiple sclerosis, Gullaine–barre syndrome, and myelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD) should be excluded.…”

Section: Introductionmentioning

confidence: 99%

Neuromyelitis optica spectrum disorder co‐existing with antiphospholipid syndrome: A case report

Edwin,

Msagati,

Komanya

et al. 2024

Clinical Case Reports

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Key clinical messageNeuromyelitis optica spectrum disorder is an autoimmune disease, rarely presents with antiphospholipid syndrome. Diagnosis and management of NMOSD are challenging in the background of diverse presentations, especially in resource‐limited settings.AbstractNeuromyelitis optica spectrum disorder (NMOSD) is a progressive demyelinating autoimmune condition resulting from the autoantibodies produced against aquaporin‐4 (AQP‐4) proteins which are widely distributed in astrocytes in the nervous system. In the setting of NMOSD, it is very crucial to consider other autoimmune diseases as differential diagnoses or co‐occurrences due to the diversity of symptoms. NMOSD co‐exists with other autoimmune diseases such as myasthenia gravis, thyroid disease, ankylosing spondylitis, pernicious anemia, thrombotic thrombocytopenic purpura, ulcerative colitis, and systemic lupus erythematosus. Few cases of antiphospholipid syndrome co‐existing with NMOSD have been reported. In resource‐limited settings, the published data are scarce, and therefore, autoimmune diseases are poorly studied. Therefore, late diagnosis and delayed treatment initiation pose long‐term sequelae and hence poor prognosis. Here, we present a case of an African woman in her early 40s presenting with bilateral progressive loss of vision, transverse myelitis, extensive longitudinal hyperintense T2 cervical lesion, and AQP‐4 autoantibody keeping with NMOSD. The co‐existence of antiphospholipid syndrome, in this case, was supported by a history of recurrent pregnancy loss and positive antiphospholipid antibodies. This case underscores the importance of individualized‐based medicine, especially in resource‐limited settings.

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Contemporary management challenges in seropositive NMOSD

Cited by 10 publications

References 72 publications

Neuromyelitis Optica Spectrum Disorders

Neuromyelitis Optica Spectrum Disorders

Clinical and epidemiological correlates of treatment change in patients with NMOSD: insights from the CIRCLES cohort

Neuromyelitis optica spectrum disorder co‐existing with antiphospholipid syndrome: A case report

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