NEW PROPHYLACTIC TREATMENTS FOR NEUROMYELITIS OPTICA SPECTRUM DISORDER WITH AQUAPORIN-4 ANTIBODIES: Α POSITION PAPER OF THE HELLENIC NEUROLOGICAL SOCIETY

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune demyelinating disease of the Central Nervous System. The diagnosis is based on the 2015 international revised criteria using clinical, laboratory, and imaging data, which is stratified further depending on the presence of aquaporin-4 (AQP4) IgG antibodies in seropositive and seronegative patients.  Until now, off-label immunosuppressive drugs have been used in NMOSD therapeutics. Recently, four new monoclonal antibodies have been approved for AQP4-IgG seropositive patients: satralizumab, eculizumab, inebilizumab, and ravulizumab. The goal of this position statement of the Neuroimmunology section of the Hellenic Neurological Society is to inform the scientific community regarding these novel therapies approved for seropositive NMOSD, suggesting an up-to-date and optimal therapeutic approach for NMOSD. In the current position paper, we summarize briefly the pathophysiology of NMOSD, the current diagnostic criteria, and the available off-label treatments. Subsequently, the newly approved therapies are presented, emphasizing on the efficacy and the main adverse reactions according to the relevant clinical trials. Finally, we provide current recommendations for the application of these individualized therapies for seropositive NMOSD patients, based on up-to-date literature.