Optimal management of neuromyelitis optica spectrum disorder with aquaporin-4 antibody using maintenance therapy with oral prednisolone (2023)

Multiple sclerosis and related diseases

Band 49,

April 2021

, 102750

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Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing neuroinflammatory disease associated with the aquaporin-4 antibody. As disability accumulates in NMOSD patients with attacks, relapse prevention is critical to improving long-term outcomes. Corticosteroids are inexpensive and promising drugs for preventing relapse in NMOSD, but few studies have evaluated the effectiveness of corticosteroids in NMOSD, particularly with regard to proper dosing and tapering.


A single-center retrospective analysis of corticosteroid therapy was performed in patients with NMOSD positive for aquaporin-4 antibodies who met the 2015 international consensus diagnostic criteria.


Medical records of a total of 89 Japanese patients with aquaporin-4 antibody-positive NMOSD treated at the Department of Neurology, Tohoku University Hospital (2000-2016) were reviewed. At the last follow-up, 66% of patients were treated with prednisolone (PSL) monotherapy, and the percentage of patients receiving PSL monotherapy or a combination of PSL and other immunosuppressants increased from 17.5% in 2000 94.1% in 2016, the annualized relapse rate (ARR) decreased from 0.78 (13 attacks in 200 person-months) in 2000 to 0.07 (5 attacks in 819 person-months) in 2016 mean ARR was significant and disabilities stabilized (PSL treatment vs. no medication, ARR: 0.21 vs. 0.98, P<0.01, change in Expanded Disability Status Scale score: +0.02 vs. +0 .89, P<0.01, observation periods: 60.1 vs. 68.2 months, P=0.26). Using Kaplan-Meier curves, the 10-year recurrence-free rate was 46.5% with PSL monotherapy and 7.1% without medication (hazard ratio: 0.069, 95% confidence interval [CI] 0.024-0.199, p<0, 01). Rapid PSL reduction (10 mg or less at 1 year and/or 5 mg or less at 2 years after clinical attacks) was compared to gradual reduction (greater than 10 mg at 1 year and greater than 5 mg at 2 years ) associated with frequent relapses after clinical attacks) (rapid versus gradual, 36.7% versus 17.7%, odds ratio 2.69, 95% CI 1.12-6.44, P=0.02). But even with PSL of 5 mg/day or less, the relapse rate after two years of acute treatment was low (before vs. after, 53.8% vs. 13.6%, odds ratio 0.12, 95% CI 0.03-0 .50, p<0.01). . Nine patients required additional immunosuppressants due to insufficient relapse prevention with PSL monotherapy. PSL monotherapy was generally well tolerated, but seven patients experienced serious adverse events, mainly fractures (5 with fracture, 1 with femoral capital necrosis, and 1 with cerebral infarction).


Our study suggests that PSL monotherapy is effective in preventing recurrence in about half of patients with aquaporin-4 antibody-positive NMOSD when doses are gradually reduced. Although it is important to have a treatment strategy tailored to each patient, this study provides evidence that PSL monotherapy may be an option for relapse prevention in some patients with NMOSD.


Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing neuroinflammatory disease associated with aquaporin immunoglobulin G (AQP4-IgG) (Lennonetal., 2005; Lennonetal., 2004). As disability accumulates in NMOSD patients with attacks, relapse prevention is critical to improving long-term outcomes. In the last decade, several studies have shown that not disease-modifying therapy for multiple sclerosis (MS), but various immunosuppressive treatments such as corticosteroids (Watanabeetal., 2007), azathioprine (Bichuettietal., 2010; Chen, H. et al. . , 2017; Costanzie tal., 2011; Elsone et. et al., 2017; Huange tal., 2018; Huhe tal., 2014; Jacobe tal., 2009; Jeonge tal., 2016; Jiao et ), methotrexate (Kitley et al., 2013), cyclosporine (Kageyamae tal., 2013; Xuetal., 2016), tacrolimus (Chen, B. et al., 2017; Kojimae tal., 2019), mitoxantrone (Enriqueze tal., 2019)22and rituximab (Damato et al., 2016; Gaoetal., 2019; Nikooetal., 2017) are effective in reducing NMOSD flare-ups. In addition, regular treatments with intravenous immunoglobulin (IVIg) (Limetal., 2020; Viswanathanetal., 2015) or plasma exchange (PE) (Khatrietal., 2012; Miyamoto and Kusunoki, 2009; Viswanathanetal., 2020) have also been reported to be effective in the Prevention of NMOSD relapse. In 2019, randomized controlled trials of therapeutics targeting the major pathogenic components of NMOSD such as: Targeted Complement CD19+ B cells) (Creeetal., 2019) have been completed and showed a remarkable effect in preventing recurrence. However, these molecularly targeted drugs are quite expensive and cannot be used in all countries and regions.

Corticosteroids, one of the most commonly used drugs worldwide to treat NMOSD, are inexpensive and promising drugs for relapse prevention (Kleiterand Gold, 2016; Trebstetal., 2014). Although many clinicians have empirically recognized the effectiveness of corticosteroids, few studies have confirmed the effectiveness of corticosteroids, particularly with regard to proper dosing and tapering.

The purpose of this study was to evaluate the efficacy of maintenance therapy with low-dose prednisolone (PSL) in preventing relapse and functional impairment in patients with AQP4-IgG-positive NMOSD. We also aimed to estimate the appropriate reduction in PSL doses following treatment of an acute exacerbation.

Extracts section


Based on the database of patients whose serological AQP4-IgG status was evaluated at Tohoku University Hospital (THU), we identified 122 AQP4-IgG positive patients who were evaluated at the TUH Neurology Clinic. Among them, patients who met the following criteria were analyzed; 1) met the 2015 international consensus diagnostic criteria for NMOSD with AQP4-IgG (Wingerchuketal., 2015), 2) was followed for more than one year from January 2000 to December 2016 at THU. AQP4 IgG

Patient brand male

A total of 94 patients met the inclusion criteria; however, four of these patients were excluded due to insufficient records and one due to participation in a clinical study. As a result, 89 patients were evaluated. The last follow-up moments were as follows: 1) December 31, 2016 (patients who were followed up in our department, n=65), 2) last visit to our department (patients who later visited other hospitals, n=14), 3 ) the date of death (n=6) and 4) the date from


This is the first study to confirm the long-term effects of PSL in a relatively large number of AQP4 IgG positive NMOSD patients. This study included patients who were followed in our unit prior to the discovery of the AQP4 antibody. In Japan, prior to the discovery of the AQP4 antibody, NMOSD was misdiagnosed as an optic spinal form of MS and it was assumed that long-term steroid therapy would not be effective in reducing MS flares. The present study showed that maintaining the PSL


We have confirmed that PSL is also useful as monotherapy to reduce relapse rate and stabilize disability in patients with AQP4 antibody-positive NMOSD. This study sheds light on how PSL therapy, including its taper, can be optimized to prevent NMOSD flare-ups. Although some monoclonal antibodies targeting immune molecules have been shown to significantly reduce the risk of NMOSD recurrence, corticosteroids are, and particularly are, inexpensive therapeutics for this disease

(Video) Neuromyelitis Optica Spectrum Disorders

Contribution statement written by CRediT

Yoshiki Takai:Conceptualization, methodology, research, resources, data maintenance, writing - original draft, visualization, project management, fundraising.Hiroshi Kuroda:Methodology, Resources, Writing - Proofreading and Editing.Tatsurou Misu:resources, supervision.Tetsuya Akaishi:methodology, validation.Ichiro Nakashima:methodology, supervision.Toshiyuki Takahashi:Investigation.Shuhei Nishiyama:Resources.Kazuo Fujihara:Resources, Writing - Proofreading and Editing, Supervision.

Declaration of Competing Interests

The authors declare that they have no known competing financial interests or personal relationships that could influence the work described in this article.


We thank Ms. Mayu Atsumi and Yukari Watanabe for technical assistance.


This study was supported in part by KAKENHI (#17K16108) from the Japanese Ministry of Education, Culture, Sports, Science and Technology (MEXT) and Scientific Research Assistance Grants from the Japanese Ministry of Health, Labor and Welfare

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    • Comparing the effect of seven drugs in preventing recurrence of neuromyelitis optica spectrum disorders: a model analysis of pooled data from the literature

      2022, International Immunopharmacology

      Neuromyelitis optica spectrum disorders (NMOSD) are an immune-mediated demyelinating disease of the central nervous system. The aim of this study was to conduct a comprehensive comparison of the effect of seven drugs in preventing NMOSD flare-ups.

      A literature search was conducted using public databases. We identified clinical trials of the seven drugs (eculizumab, inebilizumab, satralizumab, rituximab, tocilizumab, azathioprine, and mycophenolate mofetil) in preventing NMOSD flare-ups. A time-course model was constructed using time to first relapse as the primary endpoint to assess the long-term effectiveness of each drug in preventing relapse.

      24 studies with 2207 patients were included in the model analysis. The results showed that monoclonal antibody therapy can significantly increase the time to first relapse. Of all seven drugs, eculizumab can significantly prevent the patient from recurring. The estimated proportion of eculizumab-treated patients without relapse at 24 months was 98.9%.

      Based on the construction of a pharmacodynamic model over time, this study performed for the first time a comprehensive quantitative comparison of seven drugs used to treat NMOSD. These results can not only serve as a quantitative complement for the rational use of drugs in clinical practice, but also provide a pharmacodynamic reference for clinical trial design and decision-making in the future.

    • Treatment response, risk of recurrence, and clinical characteristics of Taiwanese patients with neuromyelitis optica spectrum disorder

      2022, Journal of the Formosan Medical Association

      The long-term course of disease and the effectiveness of maintenance therapies have rarely been evaluated in Asian patients with neuromyelitis optica spectrum disorder (NMOSD).

      Medical records of patients who met the 2015 International Consensus Diagnostic Criteria for NMOSD at three medical centers in Taiwan were systematically analyzed. A linear regression analysis was performed to examine factors related to annualized relapse rate (ARR); Survival analysis was used to estimate relapse-free intervals between therapies.

      A total of 557 relapses in 648 regions (202 optic neuritis, 352 acute myelitis, and 94 brain syndromes) in 204 patients were analyzed during a follow-up period of 69.5 months (range 1 to 420). Up to 36.1% of patients with onset of myelitis and 24.0% of patients with onset of optic neuritis have a localized form of the disease, defined as one or more relapses confined to the same region. The median ARR was significantly lower in patients with limited form of disease than in patients with relapses affecting multiple regions (0.30 vs. 0.47). Older age at onset was associated with lower ARR (p=0.023). Kaplan-Meier analysis showed that the estimated time (months) to the next relapse was longer in the rituximab treatment group (58.0 ± 13.2), followed by the immunosuppressant groups (48.5 ± 4.8 ) or prednisone (29.6±4.6) and lower in those without maintenance therapy (27.6±4.2) (p=8.1×10−7).

      The limited form of the disease and advanced age at onset are associated with a lower rate of recurrence in NMOSD. Compared to no maintenance therapy, rituximab and the immunosuppressant significantly reduce the risk of recurrence.

    • Exploring steroid reduction in patients with neuromyelitis optica spectrum disorder treated with satralizumab in SAkuraSky: a case series

      2022, Multiple Sclerosis and Related Disorders

      Neuromyelitis optica spectrum disorder (NMOSD) is a rare, chronic autoimmune disorder characterized by astrocytopathic lesions in the central nervous system (Beckman and others, 2019;Fujihara and others, 2020). The primary goal of NMOSD maintenance therapy is to reduce the frequency and severity of relapses and minimize future disability.Fujihara and others, 2020). Oral corticosteroids are used long-term to prevent relapse, but come with serious complications.Kessler and others, 2016;Kimbrough and others, 2012). In the SAkuraSky study, satralizumab reduced the risk of recurrence in patients with NMOSD compared to placebo, with similar rates of serious adverse events and infections between treatment arms (Yamamura et al., 2019). Here we report on 16 patients who reduced their steroid dose during SAkuraSky's open-label extension (OLE) phase.

      SAkuraSky was a phase 3, multi-center, randomized, double-blind (DB), placebo-controlled study of satralizumab in combination with immunosuppressive therapies (ISTs) in patients with NMOSD. Patients were randomized 1:1 to receive subcutaneous satralizumab 120 mg or placebo plus a stable dose of their STI at baseline. Upon completion of the DB period or after a relapse, patients may enter the OLE period. In OLE, all patients received satralizumab and actual doses can be reduced at investigator discretion. We evaluated different steroid taper patterns and their impact on relapse and safety. Patients were considered to have reduced their steroids if their steroid dose on the clinical cut-off date (CCOD: February 18, 2020) was less than on the first day of OLE. The annualized relapse rate (ARR) was calculated as the number of relapses divided by the total patient-years at risk.

      A total of 36 patients receiving oral corticosteroids entered the OLE, 16 of whom had their steroid dose reduced. The mean age (range) at baseline was 44.9 (16-73) years, all 16 were female, 14 (88%) were Japanese, and 15 (94%) were AQP4 IgG seropositive. None got additional STIs. Patients decreased their steroids from a mean of 10 (range: 5-25) mg/day at baseline OLE to 2.75 (0-15) mg/day at CCOD. Three patients discontinued steroids completely and all three remained disease free. In one patient who remained recurrence-free, the steroid dose was transiently increased. Three relapses were observed in two patients who tapered steroids during OLE; All three relapses required treatment. One of the relapses occurred shortly after the steroid dose was reduced from 25 to 10 mg/day. The ARR for patients on a steroid taper was numerically lower in the OLE phase than in the satralizumab group in the DB phase. The safety profile of satralizumab was consistent with that of the general population of SAkuraSky. Two serious infections have been observed in patients with tapering steroids in the OLE, both in the same patient: one event (hepatitis E) occurred before the patient began reducing the steroid dose; and one event (influenza) occurred while the patient was sleeping off.

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      During the SAkuraSky OLE, 16 patients reduced steroids and the ARR has not increased since the DB period. Patient numbers limit the interpretation.

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      The purpose of this study was to evaluate the use of superlatives in news articles describing treatments for multiple sclerosis.

      We searched Google News for 11 predefined superlatives describing multiple sclerosis therapies in online news articles.

      We found that news articles often describe non-FDA-approved multiple sclerosis therapies in superlatives without providing clinical evidence or attributions. In addition, no articles were published on a HONcode-certified website.

      It is important that healthcare professionals are aware of medical misinformation that is being presented to the public.

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      : Self-reported results are important for epidemiological research, but they are prone to bias.

      : In this study, we assessed: 1) the consistency and validity of the year of self-reported diagnosis among people living with MS, 2) factors associated with participant inconsistency, and 3) the impact of the observed inconsistency on participants the calculation of the Multiple Sclerosis Severity Score (MSSS).

      : Using data from the Australian MS Longitudinal Study, we assessed year-of-year consistency of self-reported diagnosis using correlation, mean differences, and percent agreement. We examined the influence of participant characteristics using Cragg constraint models and determined the effect of the observed inconsistency in the MSSS using simulations versus paired t-tests.

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      : These data support the use of the patient-reported year of diagnosis, as people with MS consistently reported their year of diagnosis and the impact of any inconsistency on clinical outcomes, such as B. the MSSS, was negligible.

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      Multiple Sclerosis and Related Disorders, Volume 49, 2021, Item 102773

      (Video) NMO/MOG

      bottom: The role of smoking in the clinical outcomes of inflammatory central nervous system (CNS) disorders is unclear. To evaluate the effect of smoking on relapses and disability in patients with neuromyelitis optica with aquaporin-4 antibodies (NMOSD-AQP4-Ab), myelin oligodendrocyte glycoprotein-associated disease (MOGAD), and patients with relapsing-remitting multiple sclerosis (MS).

      methods: in a UK cohort of 101 NMOSD-AQP4-Ab, 70 MOGAD and 159 MS and a Korean cohort of 97 NMOSD-AQ4-Ab, time to first flare, annualized flare rate, severity and recovery from first flare, time to expanded disability Status Score (EDSS)/Secondary Progressive MS (SPMS) were compared between never smokers and smokers. All clinical data were collected between January 2017 and January 2019 under local ethics.

      Results:Smoking did not affect the risk of recurrence in any of the diseases. The risk of achieving EDSS 6.0 was higher in smokers in the UK NMOSD-AQP4-Ab cohort, but this did not reach significance (HR 2.12, p=0.068). Combining the UK and Korean NMOSD-AQP4-Ab cohorts, poorer recovery from attack onset was significantly more likely than never among smokers (55% vs. 38%, p=0.04). In the MS cohort, the risk of achieving EDSS 6 and SPMS was significantly higher in smokers (HR=2.67, p=0.01 and HR=3.18, p=0.001). Similar patterns were observed in MOGAD without reaching significance.

      Conclusions: In the NMOSD-AQP4-Ab, smoking is associated with worse disability, not through increased risk of recurrence, but through poor recovery from relapse. As in MS, smoking cessation should be encouraged in NMOSD-AQP4-Ab.

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      A long-term follow-up of rituximab treatment in 20 Chinese patients with neuromyelitis optica spectrum disorders

      Multiple Sclerosis and Related Disorders, Volume 40, 2020, Article 101933

      Rituximab has been recommended as first-line therapy for neuromyelitis optica spectrum disorders (NMOSD); However, their therapy still needs to be optimized. Our aim was to present our experience in treating adult Chinese patients with NMOSD with low-dose rituximab and to further investigate its efficacy and safety in a long-term follow-up.

      Twenty adult Chinese patients with NMOSD who received rituximab injections were retrospectively analyzed and followed over a long period of time in our hospital. Efficacy was primarily assessed by the change in the expanded disability status scale and the annualized relapse rate before and after rituximab therapy. Safety was reflected by the incidence and severity of side effects.

      Rituximab treatment regimens, including doses and intervals, varied widely among patients. The median dose was 500 mg and ranged from 100 mg to 1000 mg. The median interval was 6.1 months and ranged from 2 to 18 months. After rituximab therapy, the median EDSS remained stable while the median ARR decreased by 90%. Of the 20 patients, 9 had 11 relapses, with a 55% relapse-free rate. Six cases of relapse occurred in less than 6 months since the previous rituximab injection. Seven cases occurred without repopulation of CD19+ B cells. There were 4 cases of infection including lung and urinary tract infections; The severity of the patients ranged from mild to severe.

      We have confirmed the long-term efficacy and safety of low-dose rituximab in adult Chinese patients with NMOSD. We recommend monitoring B cell repopulation to inform individualized therapies for patients with NMOSD in clinical practice.

    • research article

      To compare the efficacy and tolerability of azathioprine, mycophenolate mofetil, and lower doses of rituximab in patients with neuromyelitis optica spectrum disorder

      Journal of the Neurological Sciences, Band 385, 2018, S. 192-197

      To observe and compare the efficacy and tolerability of azathioprine (AZA), mycophenolate mofetil (MMF), and lower doses of rituximab (RTX) in patients with neuromyelitis optica spectrum disorder.

      In this prospective cohort, AQP4 IgG seropositive patients with neuromyelitis optica spectrum disorder (NMOSD) were enrolled and randomized into three groups receiving AZA, MMF, or lower doses of RTX (defined as intravenous injection of 100 mg RTX once per week for 4 consecutive weeks) or Annualized Relapse Rate (ARR), EDSS scores, peripheral blood CD19+ B-cell counts, serum AQP-4 IgG titers, and adverse drug reactions were compared between three groups.

      In the AZA group (n=22), the MMF group (n=30) and the RTX group (n=20), 54.5%, 60.0% and 65.0% of the patients achieved a relapse-free state and EDSS score improved in 90.9%, 83.3%, and 90.0% of patients, respectively. In addition, there was a significant reduction in ARR in all three groups. A reduced dose of RTX had a significant effect in reducing CD19+ B cell counts (P<0.01). Compared to the AZA group, the MMF group and the RTX group apparently lowered the AQP-4 IgG titer and caused fewer side effects. Neither the Kaplan-Meier survival curves nor the Cox proportional hazards model showed a significant difference in relapse between the three groups (P > 0.05).

      (Video) GNI Grand Rounds: The Spectrum of NMOSD (Neuromyelitis Optica Spectrum Disorder)

      AZA, MMF, and reduced doses of rituximab are effective in reducing ARR and improving clinical symptoms in patients with NMOSD. Lower RTX doses are more effective than others at reducing CD19 B cell counts. MMF and reduced RTX decrease AQP-4 IgG titer more and cause fewer side effects than AZA. However, more multicenter studies are needed to find a more effective therapeutic regimen.

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