MOGAD: A comprehensive review of clinicoradiological features, therapy and outcomes in 4699 patients globally.

Autor: Trewin BP; Translational Neuroimmunology Group, Faculty of Medicine and Health, University of Sydney, Kids Neuroscience Centre, Children's Hospital at Westmead, Sydney, Australia; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia., Brilot F; Brain Autoimmunity Group, Kids Neuroscience Centre, Kids Research at Children's Hospital at Westmead, Sydney, Australia; School of Medical Sciences and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Australia., Reddel SW; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Department of Neurology, Concord Hospital, Sydney, Australia., Dale RC; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Clinical Neuroimmunology Group, Kids Neuroscience Centre, Children's Hospital at Westmead, Sydney, Australia; TY Nelson Department of Neurology, Children's Hospital at Westmead, Sydney, Australia., Ramanathan S; Translational Neuroimmunology Group, Faculty of Medicine and Health, University of Sydney, Kids Neuroscience Centre, Children's Hospital at Westmead, Sydney, Australia; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Department of Neurology, Concord Hospital, Sydney, Australia. Electronic address: sudarshini.ramanathan@sydney.edu.au.
Jazyk: angličtina
Zdroj: Autoimmunity reviews [Autoimmun Rev] 2024 Nov 20; Vol. 24 (1), pp. 103693. Date of Electronic Publication: 2024 Nov 20.
DOI: 10.1016/j.autrev.2024.103693
Abstrakt: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is one of the most common antibody-mediated CNS disorders. Optimal diagnostic and prognostic biomarkers remain unclear. Our aim was to clarify these biomarkers and therapeutic outcomes internationally. We reviewed articles from 2007 to 2022 and identified 194 unique cohorts encompassing 4699 paediatric and adult patients from 31 countries. Where phenotypes were specified, the most common initial presentation overall was optic neuritis (ON; paediatric 34 %; adults 60 %), during which 71 % had papilloedema on fundoscopy. The most common phenotype at latest follow-up was relapsing ON (20 %). Only 47 % of patients with 6-24 months of follow-up exhibited a relapsing course, while this proportion was much higher (72 %) when follow-up was extended beyond 5 years. Despite a similar relapse rate, the time to first relapse was much shorter in paediatric than adult patients (6 vs 17 months). Adult MRI-Brain scans performed at onset were more frequently normal than in paediatric patients (50 % vs 27 %). Abnormal MRI scans showing involvement of deep grey matter, cortico-subcortical, periventricular lesions, leptomeningeal enhancement, H-shaped spinal cord lesions, and bilateral optic nerve abnormalities were more common in paediatric patients compared to adults. Conversely, adults demonstrated higher frequencies of eccentric spinal cord lesions and intraorbital involvement. CSF analysis demonstrated intrathecally restricted oligoclonal bands in 12 %, elevated protein in 35 %, and pleocytosis in 54 %. Peripapillary retinal nerve fibre layer (pRNFL) thickness, measured acutely, frequently demonstrated swelling (weighted-median 145 μm; normal 85-110). Most cohorts demonstrated notable pRNFL atrophy at latest follow-up (weighted-median 67 μm). pRNFL thickness was significantly lower when measured at or after six months following ON onset, compared to measurements taken within the first six months following ON onset (p < 0.001). Therapeutic and outcome data was available for 3031 patients with a weighted-median disease duration of 32 months. Acute immunotherapy was initiated in 97 %, and maintenance immunotherapy in 64 %, with considerable regional variation. Expanded Disability Status Scale (EDSS) scores and visual acuities improved from nadir to latest follow-up in most patients. A negative correlation was noted between follow-up pRNFL thickness and latest follow-up visual acuity (r = -0.56). Based on this unprecedented global aggregation of MOGAD patients, we reveal a higher proportion of relapsing patients than previously recognised. While commonly used measures like EDSS show significant recovery, they underestimate visual disability following optic neuritis, the most frequent clinical presentation. Our findings suggest that RNFL thickness, especially when measured at least 6 months post-ON, may serve as a more sensitive biomarker for long-term visual impairment.
Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Sudarshini Ramanathan reports financial support was provided by NHMRC Investigator Grant GNT2008339. Sudarshini Ramanathan reports a relationship with National Health and Medical Research Council that includes: funding grants. Funding BT receives a University of Sydney postgraduate scholarship and a stipend from CIA Ramanathan's Royal Australasian College of Physicians Research Establishment Fellowship. FB has received research funding from NSW Health, MS Australia, the NHMRC (Australia), the Medical Research Future Fund (Australia), The MOG Project (Apollo Grant) and Novartis. RCD has received research funding from the Star Scientific Foundation, The Trish Multiple Sclerosis Research Foundation, Multiple Sclerosis Research Australia, the Petre Foundation and the NHMRC (Australia; Investigator Grant). SR has received research funding from the National Health and Medical Research Council (NHMRC, Australia), the Petre Foundation, the Brain Foundation, the Royal Australasian College of Physicians, and the University of Sydney. She is supported by an NHMRC Investigator Grant (GNT2008339). Competing Interests BT was on an advisory board for Merck. FB was on an advisory board for Novartis and Merck, and has been an invited speaker for Biogen, Novartis and Limbic Neurology. She is on the medical advisory board (non-remunerated positions) of The MOG Project and the Sumaira Foundation. RCD received honoraria from Biogen Idec as an invited speaker, and is on the IDMC for a Roche RCT in paediatric MS. He is on the medical advisory board (non-remunerated position) of The MOG Project. SR serves as a consultant on an advisory board for UCB and Limbic Neurology and has been an invited speaker for educational/research sessions coordinated by Biogen, Alexion, Novartis, Excemed and Limbic Neurology. She is on the medical advisory board (non-remunerated positions) of The MOG Project and the Sumaira Foundation. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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