Treatment of neuromyelitis optica spectrum disordersRomeo, Andrew R.; Segal, Benjamin M.Current Opinion in Rheumatology: May 2019 - Volume 31 - Issue 3 - p 250–255 doi: 10.1097/BOR.0000000000000603 CLINICAL THERAPEUTICS & HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune Buy SDC Abstract Author InformationAuthors Article MetricsMetrics Purpose of review This review discusses concepts for diagnosing neuromyelitis optica spectrum disorders (NMOSD), distinguishing NMOSD from other inflammatory diseases of the central nervous system, and highlights recent and forthcoming data on acute and maintenance therapy of NMOSD. Recent findings The neurologic manifestations of NMOSD are heterogenous, extending beyond classic presentations of optic neuritis and longitudinally extensive transverse myelitis. NMOSD may be comorbid with rheumatologic diseases, such as systemic lupus erythematosus, but is recognized as a distinct entity. Recent studies of acute treatment of NMOSD support early use of plasmapheresis. Relapse prevention is essential, as relapses can be disabling and patients may have only partial recovery. Current practice generally recommends at least 5 years of maintenance treatment. Recent randomized data demonstrates superiority of rituximab over azathioprine. Phase 3 trials have recently been completed or are underway studying novel therapies employing B-cell depletion, complement inhibition, and cell-based mechanisms (among other mechanisms) for maintenance therapy of NMOSD. Summary NMOSD is a heterogeneous but well-defined clinical entity, distinct from other neurologic and systemic inflammatory diseases, and treatment is poised for expansion. Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA Correspondence to Andrew R. Romeo, MD, Department of Neurology, University of Michigan, 1500 E. Medical Center Drive, Floor 1, Reception C, Ann Arbor, MI 48109, USA. Tel: +1 734 232 0438; e-mail: firstname.lastname@example.org Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.