Emerging Targeted Therapies for Neuromyelitis Optica Spectrum Disorders

AQP4‐IgG positive paraneoplastic NMOSD: A case report and review

“…The immunotherapy mainly includes immunosuppressive drugs in combination with low-dose corticosteroids (Holmøy et al, 2020). Several new drugs, such as eculizumab, satralizumab, and inebilizumab, have demonstrated efficacy in phase III clinical trials targeting the maintenance phase of NMOSD (Valencia-Sanchez & Wingerchuk, 2021).…”

“…The immunotherapy mainly includes immunosuppressive drugs in combination with low‐dose corticosteroids (Holmøy et al., 2020 ). Several new drugs, such as eculizumab, satralizumab, and inebilizumab, have demonstrated efficacy in phase III clinical trials targeting the maintenance phase of NMOSD (Valencia‐Sanchez & Wingerchuk, 2021 ). Theoretically, there is a possibility that immunosuppression may lead to tumor progression, but there is no clear evidence, and immunosuppression may have some benefits in improving nervous system damage, so there is no consensus on whether immunosuppressive agents should be used in patients with paraneoplastic NMOSD.…”

Neuromyelitis optica spectrum disorders (NMOSD) and systemic lupus erythematosus (SLE): Dangerous duo

“…1,21,24 Since 2019, the therapeutic landscape for NMOSD has been transformed with the introduction of more targeted therapy and with the FDA approval of 3 new drugs namely Eculizumab (terminal complement protein C5 inhibitor), Inebilizumab (CD-19 directed cytolytic antibody) and Satralizumab (IL-6 inhibitor) all demonstrating robust benefits in preventing relapses. 1,4,25 Modulation of the complement system in the treatment of NMOSD was proposed with Eculizumab which was the first FDAapproved treatment for adults with AQP4 antibody-positive NMOSD. 25,26 Eculizumab is a monoclonal antibody (an anti-C5 complement inhibitor) that specifically binds to the complement component C5, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-C9.…”

Glial cells and neurologic autoimmune disorders

“…Neuromyelitis optica spectrum disorders are destructive inflammatory diseases of the CNS induced by autoantibodies, often causing demyelination of the CNS, mainly affecting the spinal cord, optic nerve, and brainstem. The typical manifestations are recurrent optic neuritis, longitudinal generalized myelitis, brainstem, diencephalon, and brain syndrome ( Valencia-Sanchez and Wingerchuk, 2021 ). For many years, optic neuromyelitis has been considered a type of MS. Until 2004, researchers found that the main pathogenic autoantibody of NMODS in patients with optic neuromyelitis was AQP4 immunoglobulin G (AQP4-IgG) targeting AQP4 on the foot process membrane of astrocytes.…”