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Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder.

Abstract：

OBJECTIVE:Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments. METHODS:We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy. RESULTS:Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5). CONCLUSION:This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.

journal_name

Neurology

journal_title

Neurology

authors

Chen JJ,Flanagan EP,Bhatti MT,Jitprapaikulsan J,Dubey D,Lopez Chiriboga ASS,Fryer JP,Weinshenker BG,McKeon A,Tillema JM,Lennon VA,Lucchinetti CF,Kunchok A,McClelland CM,Lee MS,Bennett JL,Pelak VS,Van Stavern G,Adesina

doi

10.1212/WNL.0000000000009758

subject

Has Abstract

pub_date

2020-07-14 00:00:00

pages

e111-e120

issue

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000009758

journal_volume

pub_type

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Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder.