Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies

Abstract

ObjectiveTo evaluate the outcomes of immunosuppressive therapy (IST) discontinuation in patients with neuromyelitis optica spectrum disorder (NMOSD) after a sustained remission period.MethodsWe retrospectively reviewed the medical records of 17 patients with antiaquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period of ≥3 years.ResultsIST was discontinued at a median age of 40 years (interquartile range [IQR], 32–51) after a median relapse-free period of 62 months (IQR, 52–73). Among the 17 enrolled patients, 14 (82%) relapsed at a median interval of 6 months (IQR, 4–34) after IST discontinuation, 3 (18%) of whom experienced severe attacks; notably, all 3 of these patients had a history of severe attack before IST. These 3 patients received steroids, followed by plasma exchange for acute treatment, but 2 exhibited poor recovery and significant disability worsening at 6 months after relapse.ConclusionsIST discontinuation may increase the risk of relapse in seropositive patients with NMOSD even after 5 years of remission. Given the potentially devastating consequence of a single attack of NMOSD, caution is advised with IST discontinuation, particularly in patients with severe attack before IST.

COVID-19 susceptibility and outcomes among patients with neuromyelitis optica spectrum disorder (NMOSD): A systematic review and meta-analysis

“…Notwithstanding the aforementioned disadvantages, the efficacy of rituximab is higher than azathioprine and MMF ( Huang et al, 2019 ; Nikoo et al, 2017 ). Moreover, drug discontinuation could predispose NMOSD patients to potentially-life-threatening attacks ( Kim et al, 2021 ; Zhao-Fleming et al, 2021 ). Therefore, the administration of rituximab should be weighed against the risk of severe infection on a case-by-case basis.…”

“…Current data suggest as an explanation that race-ethnicity minority populations experience social and financial inequity and have inadequate access to healthcare services (Kabarriti et al, 2020;Ogedegbe et al, 2020) One of the greatest challenges physicians are facing during the pandemic is managing immunosuppressive therapies in patients with autoimmune diseases. NMOSD in most cases follows a relapsing course and may lead to neurological disability and morbidity as a consequence of cumulative sequelae of attacks, justifying early initiation of immunosuppressive treatment (Kim et al, 2021;Zhao-Fleming et al, 2021). Azathioprine and mycophenolate mofetil are two common immunosuppressive agents used for the treatment of NMOSD patients.…”

Clinical Analysis of NMOSD with Area Postrema Syndrome as Initial Symptom

“…Older age, shorter time between onset and rst onset, and lack of Immunosuppressive therapy (IST) have been found to be associated with higher mortality, regardless of the severity of the onset [32]. According to the follow-up of 17 patients with NMOSD, 14 of them relapsed after six months of drug withdrawal, suggesting that patients with severe clinical symptoms before IST treatment should be carefully discontinued [33]. A Chinese single-center study also supports the view that more caution is needed when discontinuing IST, regardless of the duration of use [34].…”

Network Meta-analysis of Food and Drug Administration-approved Treatment Options for Adults with Aquaporin-4 Immunoglobulin G-positive Neuromyelitis Optica Spectrum Disorder

“…An estimated 80-90% of NMOSD cases follow a relapsing disease course [1,6]. Any relapse can result in the accumulation of neurological disability, including blindness and paralysis, which highlights the immediate need for immunotherapies that effectively prevent NMOSD relapses [7][8][9][10][11][12][13].…”