SPHERES: innovative registry addressing the new era of neuromyelitis optica spectrum disorders (NMOSD) therapy.

Neuromyelitis Optica Spectrum Disorders (NMOSD) are rare, autoimmune disorders of the central nervous system (CNS) that affect optic nerves and spinal cord. The SPHERES (Synergy of Prospective Health & Experimental Research for Emerging Solutions) Registry for NMOSD, a prospective observational cohort of adult patients in the United States, established in June 2021, was designed to gather robust and high-quality longitudinal data to facilitate knowledge of NMOSD and better understand the newly approved therapeutics. The objective of this study was to detail the SPHERES Registry features and characterize the SPHERES population in aggregate and stratified by serostatus at registry enrollment. Registry questionnaires were completed by clinicians and patients at enrollment and at routine clinical visits occurring at ∼6-month intervals, with additional data collection between visits. At enrollment into SPHERES, mean age of the first 350 patients enrolled was 50.1 years, 84 % of the cohort was female, 66 % had anti-AQP4+ NMOSD, and 71 % reported use of a biologic therapy. A number of demographic and disease characteristics and disease activity measures varied by serostatus, including mean durations since symptom onset and diagnosis and mean EDSS score. Patient-reported outcome measures were generally similar across serostatus groups. This analysis of the initial SPHERES patients provides valuable insights into the ability of the Registry to capture the natural history, real-world effectiveness, therapeutic utilization and safety in treating NMOSD.