Epidemiology of NMOSD in Catalonia: Influence of the new 2015 criteria in incidence and prevalence estimates

Neuromyelitis optica spectrum disorders (NMOSD) are autoantibody mediated chronic inflammatory diseases. Serum antibodies (Abs) against the aquaporin-4 water channel lead to recurrent attacks of optic neuritis, myelitis and/or brainstem syndromes. In some patients with symptoms of NMOSD, no AQP4-Abs but Abs against myelin-oligodendrocyte-glycoprotein (MOG) are detectable. These clinical syndromes are now frequently referred to as “MOG-encephalomyelitis” (MOG-EM). Here we give an overview on current recommendations concerning diagnosis of NMOSD and MOG-EM. These include antibody and further laboratory testing, MR imaging and optical coherence tomography. We discuss therapeutic options of acute attacks as well as longterm immunosuppressive treatment, including azathioprine, rituximab, and immunoglobulins.

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“…Using the 2015 instead of the 2006 criteria led to a significant increase in the number of patients diagnosed with NMOSD ( 138 , 171 , 172 )…”

Section: Diagnosismentioning

confidence: 99%

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

et al. 2018

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“…Neuromyelitis optica (NMO) is an inflammatory disease affecting the CNS ( 14 ) with similar physiopathology as MS, but is considered an autoimmune astrocytopathy. NMO is a rare disease which presents with incidence between 0.05 and 0.4/100,000 ( 15 , 16 ). About 70% of the patients diagnosed with NMO shows the presence of anti-aquaporin-4 (AQP4) antibody as well as specific T-lymphocytes in the bloodstream or CSF which suggest the pro-inflammatory role of these cells ( 17 ).…”

Section: Introductionmentioning

confidence: 99%

Tolerogenic Dendritic Cells as a Promising Antigen-Specific Therapy in the Treatment of Multiple Sclerosis and Neuromyelitis Optica From Preclinical to Clinical Trials

Flórez‐Grau

Zubizarreta

Cabezón³

et al. 2018

Front. Immunol.

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The identification of activated T-lymphocytes restricted to myelin-derived immunogenic peptides in multiple sclerosis (MS) and aquaporin-4 water channel in neuromyelitis optica (NMO) in the blood of patients opened the possibility for developing highly selective and disease-specific therapeutic approaches. Antigen presenting cells and in particular dendritic cells (DCs) represent a strategy to inhibit pro-inflammatory T helper cells. DCs are located in peripheral and lymphoid tissues and are essential for homeostasis of T cell-dependent immune responses. The expression of a particular set of receptors involved in pathogen recognition confers to DCs the property to initiate immune responses. However, in the absence of danger signals different DC subsets have been revealed to induce active tolerance by inducing regulatory T cells, inhibiting pro-inflammatory T helper cells responses or both. Interestingly, several protocols to generate clinical-grade tolerogenic DC (Tol-DC) in vitro have been described, offering the possibility to restore the homeostasis to central nervous system-related antigens. In this review, we discuss about different DC subsets and their role in tolerance induction, the different protocols to generate Tol-DCs and preclinical studies in animal models as well as describe recent characterization of Tol-DCs for clinical application in autoimmune diseases and in particular in MS and NMO patients. In addition, we discuss the clinical trials ongoing based on Tol-DCs to treat different autoimmune diseases.

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“…Increased diagnostic accuracy and increased health care provider awareness have resulted in increased prevalence up to 10/100,000 in some geographic regions. [5][6][7][8] This estimate equates to >15,000 US patients and >100,000 cases worldwide. NMOSD disproportionately affects females (up to 7:1).…”

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confidence: 99%

Neuromyelitis optica spectrum disorder

Beekman

Keisler

Pedraza

et al. 2019

Neurol Neuroimmunol Neuroinflamm

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ObjectiveTo gain insights into NMOSD disease impact, which may negatively affect QoL of patients, their families, and social network.MethodsThe current study used validated instruments to assess physical, emotional, and socioeconomic burden of NMOSD on QoL among 193 patients.ResultsA majority of patients reported an initial diagnosis of a disease other than NMOSD. Overall, two-thirds of patients reported NMOSD as having a strong negative impact on physical health (Short Form-36 [SF-36] score 27.1 ± 39.1), whereas emotional well-being was relatively unimpaired on average (SF-36 score 54.0 ± 44.9). A subset of patients reported having the highest category of emotional health despite worse physical health or financial burden, suggesting psychological resilience. Pain (r = 0.61) and bowel/bladder dysfunction (r = 0.41) imposed the greatest negative physical impact on overall QoL. In turn, ability to work correlated inversely with worsened health (r = −0.68). Increased pain, reduced sexual function, inability to work, and reduced QoL had greatest negative impacts on emotional well-being. Dissatisfaction with treatment options and economic burden correlated inversely with QoL.ConclusionsCollectively, the current findings advance the understanding of physical, emotional, social, and financial tolls imposed by NMOSD. These insights offer potential ways to enhance QoL by managing pain, enhancing family and social networks, and facilitating active employment.

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Epidemiology of NMOSD in Catalonia: Influence of the new 2015 criteria in incidence and prevalence estimates

Abstract: The new criteria increase the estimates, but NMOSD remains as a rare disease. The differences in age- and sex-specific estimates highlight the importance of the serologic classification.

Cited by 82 publications

References 19 publications

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

Tolerogenic Dendritic Cells as a Promising Antigen-Specific Therapy in the Treatment of Multiple Sclerosis and Neuromyelitis Optica From Preclinical to Clinical Trials

Neuromyelitis optica spectrum disorder

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