PMID- 35839615 OWN - NLM STAT- MEDLINE DCOM- 20221019 LR - 20221019 IS - 2211-0356 (Electronic) IS - 2211-0348 (Linking) VI - 66 DP - 2022 Oct TI - Treatment outcomes of first-ever episode of severe optic neuritis. PG - 104020 LID - S2211-0348(22)00528-4 [pii] LID - 10.1016/j.msard.2022.104020 [doi] AB - BACKGROUND: Severe optic neuritis (ON) is an acute inflammatory attack of the optic nerve(s) leading to severe visual loss that may occur in isolation or as part of a relapsing neuroinflammatory disease, such neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD), or more rarely multiple sclerosis (MS). In cases of first-ever severe ON of uncertain etiology best treatment strategies remain unclear. METHODS: We reviewed records of all patients with a documented diagnosis of ON between 2004 and 2019 at Mass General Brigham (MGB) and Johns Hopkins University (JHU) hospitals. Out of 381 patients identified, 90 (23.6%) satisfied the study criteria for severe ON with visual acuity (VA) equal to or worse than 20/200 (logMAR=1) at nadir in the affected eye and had sufficient follow-up data. Treatment strategies with corticosteroids only or treatment escalation with therapeutic plasma exchange (PLEX) after steroids were compared and evaluated for differences in visual outcomes at follow-up. RESULTS: Of the 90 patients with severe optic neuritis, 71(78.9%) received corticosteroids only, and 19 (17.0%) underwent PLEX following corticosteroids. Of the 71 patients who received steroids without escalation to PLEX, 30 patients (42.2%) achieved complete recovery (VA 20/20 on the affected eye), whereas 35 (49.3%) had a partial recovery and 6 (8.4%) had no recovery. Among the 19 corticosteroid non-responders patients who underwent escalation treatment, 13 (68.4%) made complete recovery, 6 (31.6%) had partial visual recoveries (p=0.0434). The median delta logMAR of patients who underwent escalation of care was -1.2 compared with 2.0 for the ones who did not (p=0.0208). A change of delta logmar 2.0 is equivalent of going from hand motion to light perception and the positive delta value refers to intra-attack worsening. Other than not responding to steroids, patients who underwent PLEX tended to have more severe ON with significantly worse nadir visual acuity compared with those who received corticosteroids alone (logMAR 3.12 (min 2.0 - max 5.0) vs. 2.17 (min 1.3 - max 3.0); p=0.004). CONCLUSION: In our cohort of first-ever severe optic neuritis of unknown etiology, patients that did not respond adequately to corticosteroids benefited from treatment escalation to PLEX, followed in most cases by Rituximab, regardless of final etiology. Randomized controlled trials are needed to confirm the best treatment strategies. CI - Copyright (c) 2022. Published by Elsevier B.V. FAU - Galetta, Kristin AU - Galetta K AD - Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. Electronic address: kgaletta@partners.org. FAU - Ryan, Sophia AU - Ryan S AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Department of Neurology, Mount Sinai Health System, New York, NY; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Manzano, Giovanna AU - Manzano G AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Chibnik, Lori B AU - Chibnik LB AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Epidemiology, Harvard T.H. Chan School of Public Health, Boston MA; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Balaban, Denis AU - Balaban D AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Prasad, Sashank AU - Prasad S AD - Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Chwalisz, Bart K AU - Chwalisz BK AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, MA; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Salazar-Camelo, Andrea AU - Salazar-Camelo A AD - Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Conway, Sarah AU - Conway S AD - Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Levy, Michael AU - Levy M AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. FAU - Matiello, Marcelo AU - Matiello M AD - Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA; Icahn School of Medicine at Mount Sinai, New York, NY; Department of Neurology, Mayo Clinic Alix School of Medicine, Rochester MN. LA - eng PT - Journal Article PT - Review DEP - 20220702 PL - Netherlands TA - Mult Scler Relat Disord JT - Multiple sclerosis and related disorders JID - 101580247 RN - 0 (Aquaporin 4) RN - 0 (Autoantibodies) RN - 0 (Myelin-Oligodendrocyte Glycoprotein) RN - 0 (Steroids) RN - 4F4X42SYQ6 (Rituximab) SB - IM MH - Aquaporin 4 MH - Autoantibodies MH - Humans MH - Myelin-Oligodendrocyte Glycoprotein MH - *Neuromyelitis Optica/complications MH - *Optic Neuritis/diagnosis MH - Retrospective Studies MH - Rituximab MH - Steroids MH - Treatment Outcome OTO - NOTNLM OT - MOGAD OT - NMOSD OT - Optic neuritis OT - Plasmapheresis COIS- Declaration of Competing Interest Dr. Galetta has received research support from the Sumaira Foundation and personal compensation for consulting from Glaxo Smith Kline unrelated to this project Dr. Ryan has no relevant disclosures Dr. Manzano has no relevant disclosures Dr. Chibnik has no relevant disclosures Dr. Balaban has no relevant disclosures Dr. Salazar-Camelo has no relevant disclosures Dr. Conway has no relevant disclosures Dr. Prasad has no relevant disclosures Dr. Chwalisz has no relevant disclosures Dr. Levy has received consulting fees and/or research grants from Alexion, Horizon, Genentech, UCB and Sanofi. Dr Matiello has received consulting fees as a member of the advisory board of Alexion EDAT- 2022/07/16 06:00 MHDA- 2022/10/20 06:00 CRDT- 2022/07/15 18:22 PHST- 2022/05/19 00:00 [received] PHST- 2022/06/24 00:00 [revised] PHST- 2022/07/01 00:00 [accepted] PHST- 2022/07/16 06:00 [pubmed] PHST- 2022/10/20 06:00 [medline] PHST- 2022/07/15 18:22 [entrez] AID - S2211-0348(22)00528-4 [pii] AID - 10.1016/j.msard.2022.104020 [doi] PST - ppublish SO - Mult Scler Relat Disord. 2022 Oct;66:104020. doi: 10.1016/j.msard.2022.104020. Epub 2022 Jul 2.