New insights into the use of high dose corticosteroids and plasmapheresis in persons with MOGAD and NMOSD

Abstract

Background: Anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD) and neuromyelitis optica spectrum disease (NMOSD) are antibody mediated diseases characterized by neurological symptoms including recurrent relapses of optic neuritis and/or myelitis, as well as other less frequent syndromes. The current treatment for acute attacks of NMOSD/MOGAD are based on clinical studies for other demyelinating diseases(i.e. Multiple Sclerosis). In NMOSD, high dose corticosteroids (HDS) are considered the standard first line therapy, with emerging evidence supporting the use of plasmapheresis (PLEX) as an acute therapy. In MOGAD, being a relatively new clinical syndrome, the consensus on acute treatments is yet to be reached. The objective of our study was to assess the efficacy of treatment regimens (no treatment vs. HDS vs. HDS and PLEX) on disability outcomes in persons with NMOSD and MOGAD-optic neuritis and myelitis.

Methods: We retrospectively extracted data from the MuSicaL-NeMo database using a mixed Natural Language Processing followed by investigators verification. We assessed the change in Expanded Disability Status Scale (EDSS) and Visual Acuity (VA) following HDS and PLEX, in persons with MOGAD and NMOSD following myelitis and optic neuritis. We used the novel statistical measure Wilcoxon-Mann-Whitney Odd (WMW-Odd) to calculate the change through all the spectrum of each ordinal scale (VA and EDSS).

Results: Eleven myelitis and 12 optic neuritis in 22 persons with MOGAD and 30 myelitis and 12 optic neuritis in 20 persons with NMOSD were included(15 Aquaporin-4 seropositive). In persons with MOGAD-optic neuritis the group receiving HDS had a WMW-Odd of 15.33(p ≤ 0.001), however those not receiving treatment also tended to improve (WMW-Odd=3.17, p = 0.06). NMOSD-optic neuritis treated with HDS only improve 33.3 % of the times (p=NS). Persons with MOGAD-myelitis receiving HDS significantly improved (WMW-Odd=7.33, p = 0.002). Persons with NMOSD-myelitis treated with HDS had an WMW-Odd of 2.56 (p = 0.002) and those treated with PLEX plus HDS (PLEX+), had similar WMW-Odd of 2.51 (p = 0.03). When correcting for disease severity by restricting inclusion to persons with NMOSD with EDSS≥4, both treatments showed a higher WMW-Odd, however the group receiving HDS continued to show higher WMW-Odd than the PLEX+ group(WMW-Odd= 3.75, p = 0.002 vs. WMW-Odd =3.05, p = 0.02, respectvely) CONCLUSION: Our study suggests that persons with MOGAD-optic neuritis improve without acute treatments, however they have very marked improvement when using HDS, as previously suggested. Patient with MOGAD-myelitis are also very responsive to HDS, however, as compared to MOGAD-optic neuritis, they displayed less improvement, if not treated. In the NMOSD group the use of PLEX in addition to HDS did not demonstrate any significant difference in EDSS outcomes. Contrary to previous suggestions, when adjusting for group differences (by only including EDSS ≥4), the use of HDS and PLEX+ did not show better results than the group using HDS.

Keywords: Acute treatment; Anti-myelin oligodendrocyte glycoprotein associated disease; High dose corticosteroids; Neuromyelitis optica spectrum disorder; Plasmapheresis.