Highlights
- •Severe optic neuritis is seen in disorders including neuromyelitis optica spectrum disorder
- •Acute treatment for patients with severe optic neuritis of unclear etiology is not clear
- •Patients with severe optic neuritis of unknown cause may benefit from treatment escalation to PLEX
Abstract
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.
Keywords
Abbreviations:
IQR (Interquartile range), logMAR (Logarithm of the minimum angle of resolution), max (maximum), min (minimum), MOGAD (Myelin oligodendrocyte antibody associated disease), NMOSD (Neuromyelitis optics spectrum disorder), ON (Optic Neuritis), PLEX (Plasma exchange)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: July 02, 2022
Accepted:
July 1,
2022
Received in revised form:
June 24,
2022
Received:
May 19,
2022
Identification
Copyright
© 2022 Elsevier B.V. All rights reserved.
