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Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder.

Sherman E, Han MH - Curr Treat Options Neurol (2015)

Bottom Line: Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM).Azathioprine, mitoxantrone, and methotrexate also have retrospective case series data that demonstrate reduction in ARR and stabilization of EDSS but with higher relapse rates and exposure to greater risk of treatment toxicities.Excitingly, multiple novel therapies are under clinical study for patients who are refractory to these first-line therapies including monoclonal antibodies targeting interleukin-6 (IL-6), CD19, CD20, complement, and neutrophil elastase inhibitors which may provide additional options for patients with severe clinical presentations.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Neurological Sciences, Stanford University, 1201, Welch Road, Stanford, CA, 94305, USA.

ABSTRACT

Opinion statement: Neuromyelitis optica and neuromyelitis optica spectrum disorder (NMO/NMOSD) is a rare but clinically aggressive demyelinating disease of the central nervous system (CNS) caused by antibodies against water channel protein aquaporin 4 (AQP4) in the astrocytic foot processes. Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM). The majority of patients with NMOSD show good response to treatment with steroids and plasmapheresis in the acute setting; however, 90 % of patients will eventually have clinical relapses and accrue permanent disability. Currently, immune modulation is the mainstay of maintenance therapy with anti CD-20 (rituximab, Rituxan™) having collectively the strongest evidence to support its use and mycophenolate mofetil having comparable reductions in absolute relapse rate (ARR) and expanded disability status scale (EDSS) scores. Azathioprine, mitoxantrone, and methotrexate also have retrospective case series data that demonstrate reduction in ARR and stabilization of EDSS but with higher relapse rates and exposure to greater risk of treatment toxicities. Excitingly, multiple novel therapies are under clinical study for patients who are refractory to these first-line therapies including monoclonal antibodies targeting interleukin-6 (IL-6), CD19, CD20, complement, and neutrophil elastase inhibitors which may provide additional options for patients with severe clinical presentations. Importantly, no randomized clinical trials have been published to date comparing clinical outcomes of different maintenance therapies in NMOSD. Several trials are currently underway, and results will help guide future management decisions as current evidence is from many small, retrospective case series and cohort studies with many potential confounds.

No MeSH data available.


Related in: MedlinePlus

Treatment algorithm for acute and chronic management of NMOSD.
© Copyright Policy - OpenAccess
Related In: Results  -  Collection


getmorefigures.php?uid=PMC4592697&req=5

Fig1: Treatment algorithm for acute and chronic management of NMOSD.

Mentions: These studies are limited by retrospective, case series design, small sample size, and multiple confounders. Head-to-head randomized trials to compare these agents are needed in the future as agents vary greatly in terms of cost. Please refer to Table 1 for a summary of common medications used in chronic management of NMOSD including dosing and side effects. Figure 1 summarizes our recommended treatment algorithm based upon current literature.Table 1


Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder.

Sherman E, Han MH - Curr Treat Options Neurol (2015)

Treatment algorithm for acute and chronic management of NMOSD.
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4592697&req=5

Fig1: Treatment algorithm for acute and chronic management of NMOSD.
Mentions: These studies are limited by retrospective, case series design, small sample size, and multiple confounders. Head-to-head randomized trials to compare these agents are needed in the future as agents vary greatly in terms of cost. Please refer to Table 1 for a summary of common medications used in chronic management of NMOSD including dosing and side effects. Figure 1 summarizes our recommended treatment algorithm based upon current literature.Table 1

Bottom Line: Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM).Azathioprine, mitoxantrone, and methotrexate also have retrospective case series data that demonstrate reduction in ARR and stabilization of EDSS but with higher relapse rates and exposure to greater risk of treatment toxicities.Excitingly, multiple novel therapies are under clinical study for patients who are refractory to these first-line therapies including monoclonal antibodies targeting interleukin-6 (IL-6), CD19, CD20, complement, and neutrophil elastase inhibitors which may provide additional options for patients with severe clinical presentations.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Neurological Sciences, Stanford University, 1201, Welch Road, Stanford, CA, 94305, USA.

ABSTRACT

Opinion statement: Neuromyelitis optica and neuromyelitis optica spectrum disorder (NMO/NMOSD) is a rare but clinically aggressive demyelinating disease of the central nervous system (CNS) caused by antibodies against water channel protein aquaporin 4 (AQP4) in the astrocytic foot processes. Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM). The majority of patients with NMOSD show good response to treatment with steroids and plasmapheresis in the acute setting; however, 90 % of patients will eventually have clinical relapses and accrue permanent disability. Currently, immune modulation is the mainstay of maintenance therapy with anti CD-20 (rituximab, Rituxan™) having collectively the strongest evidence to support its use and mycophenolate mofetil having comparable reductions in absolute relapse rate (ARR) and expanded disability status scale (EDSS) scores. Azathioprine, mitoxantrone, and methotrexate also have retrospective case series data that demonstrate reduction in ARR and stabilization of EDSS but with higher relapse rates and exposure to greater risk of treatment toxicities. Excitingly, multiple novel therapies are under clinical study for patients who are refractory to these first-line therapies including monoclonal antibodies targeting interleukin-6 (IL-6), CD19, CD20, complement, and neutrophil elastase inhibitors which may provide additional options for patients with severe clinical presentations. Importantly, no randomized clinical trials have been published to date comparing clinical outcomes of different maintenance therapies in NMOSD. Several trials are currently underway, and results will help guide future management decisions as current evidence is from many small, retrospective case series and cohort studies with many potential confounds.

No MeSH data available.


Related in: MedlinePlus