Neuromyelitis optica: Clinical course and potential prognostic indicators.

Autor: Masha N; Duke University School of Medicine, 8 Searle Center Dr, Durham, NC 27710, USA. Electronic address: nidhila.masha@duke.edu., Kimbrough DJ; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Eckstein CP; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Hudak NM; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Skeen MB; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Hartsell FL; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Lutz MW; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA., Shah S; Department of Neurology, Duke University Medical Center, 40 Duke Medicine Cir Clinic 1L, Durham, NC 27710, USA.
Jazyk: angličtina
Zdroj: Multiple sclerosis and related disorders [Mult Scler Relat Disord] 2023 Jan; Vol. 69, pp. 104414. Date of Electronic Publication: 2022 Nov 19.
DOI: 10.1016/j.msard.2022.104414
Abstrakt: Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune neurological disorder associated with antibodies to aquaporin-4 (AQP4). NMOSD has been thought to follow a progressive disease course, with step-wise accumulation of disability over time, even in patients undergoing immunosuppressive/immunomodulatory therapy. The influence of factors such as AQP4 seropositivity, AQP4 serum titer levels, and administration of plasmapheresis on NMOSD prognosis is, as yet, unclear.
Methods: We performed a retrospective chart review of 53 persons with NMOSD at Duke University Hospital-collecting data on longitudinal disease course, imaging, demographics, and serum AQP4 titers (measured using the ELISA or FACS method). Most patients in our cohort were treated with high-dose corticosteroids and, following diagnosis, received maintenance immunosuppressive/immunomodulatory therapies. Longitudinal data on EDSS scores were used to calculate the slope of disability over time for each participant. We additionally investigated the correlation between initial AQP4 seropositivity, initial AQP4 serum titer levels, and treatment with plasmapheresis on disability progression for each participant.
Results: Contrary to current views on NMOSD disease course, the majority of our participants showed either no change (31.9%) or improvement (27.1%) in disability over time. Our results additionally revealed no significant association between clinical prognosis and initial AQP4 seropositivity (p = 0.830), initial AQP4 serum titer levels (p = 0.338), or administration of plasmapheresis (p = 0.1149).
Conclusions: Our study presents a contemporary view of the clinical course of NMOSD and shows a more favorable view of its disease course than prior studies (performed before high-efficacy disease modifying therapies became widely-used for this patient population). Most patients in this study received treatment with high-dose corticosteroids following NMOSD flares, as well as a variety of maintenance immunosuppressive therapies. The results of this study cannot shed light on the disease course of untreated NMOSD. Our findings additionally challenge the theory that AQP4 seropositivity or serum titer levels at time of diagnosis may be used to effectively predict NMOSD prognosis. While we were unable to find evidence supporting a favorable effect of plasmapheresis administration on disease outcomes, further research is needed to determine the role plasmapheresis ought to play in the treatment of NMOSD.
Competing Interests: Declaration of Competing Interest Suma Shah receives research support from Verasci and Biogen. She has received honaria from EMD Serono. Dorlan Kimbrough serves as a consultant for CVS Health Christopher P. Eckstein receives research funding from Sanofi, Genzyme, and EMD Serono—as well as honoraria from Viela Bio. Mark B. Skeen serves as a consultant for Biogen, Novartis, Alexion, and Brixtol Myers Squibb, and works part-time at WCG. Michael W. Lutz receives funding for his research from NIA/NIH. Dr. Lutx received consulting fees and travel expenses to attend scientific conferences from Zinfandel Pharmaceuticals. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors
(Copyright © 2022. Published by Elsevier B.V.)
Databáze: MEDLINE
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