Efficacy and Safety of Plasma Exchange in Neuromyelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody Disease in Adults: A Systematic Review and Meta-analysis
Marina Vilardo1, Bruna Leles Vieira de Souza1, Joao Vitor Mahler1, Leonardo Cardoso2, Marcelo Matiello3
1Neurology, Massachusetts General Hospital, 2Universidade Federal de Minas Gerais, 3Massachusetts General Hospital, Brigham, Harvard
Objective:
To analyze the efficacy and safety of therapeutic plasma exchange (PLEX) for neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD) acute attack.
Background:
NMOSD and MOGAD are autoimmune demyelinating disorders of the central nervous system usually treated with intravenous methylprednisolone (IVMP) for acute attacks. PLEX has increasingly been recognized as rescue therapy or even first-line therapy, although comparison studies are scarce and there is still no consensus.
Design/Methods:
PubMed, Embase, Web-of-Science, and Cochrane were systematically searched according to PRISMA guidelines. Studies were eligible if they reported Expanded Disability Status Scale (EDSS), visual acuity (logMAR) or adverse events (AE) after PLEX treatment and at 6-months follow-up for acute attacks of NMOSD or MOGAD in adults. Pooled mean difference (MD) was calculated using the random-effects model.
Results:
27 studies (22 retrospective, 4 prospective, 1 randomized controlled trial), comprising 1,170 patients (80% female; mean age 40.8 ± 13.4 years) with 1,128 NMOSD attacks (73% AQP4 seropositive) and 99 MOGAD attacks. PLEX was given after IVMP in 73%, simultaneously with IVMP in 15%, and alone in 3% of patients, respectively. In terms of efficacy, EDSS decreased after PLEX (MD=0.85, 95% CI 0.48–1.22, p<0.001) and continued to improve at follow-up (MD=2.34, 1.67–3.01, p<0.0001). LogMAR improved immediately (MD=0.75, 0.38–1.12, p=0.001) and at follow-up (MD = 1.22, 0.88–1.55, p<0.00001). The subanalyses according to PLEX administration and antibody status showed consistent improvement, favoring PLEX given after IVMP and AQP4-IgG–positive subgroup. Regarding safety, adverse events occurred in 22% (13–35%, p<0.01) and serious adverse events in 2% (1–4%, p=0.08). All complications were successfully managed and there were no procedure-related deaths.
Conclusions:
This large-scale systematic review and meta-analysis demonstrated the benefit of PLEX for NMOSD and MOGAD attacks with significant improvements in disability and vision both immediately and at ≥6 months, with low pooled rates of serious complications and no mortality.
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