Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder

John J. Chen*, Eoin P. Flanagan, M. Tariq Bhatti, Jiraporn Jitprapaikulsan, Divyanshu Dubey, Alfonso (sebastian) S. Lopez Chiriboga, James P. Fryer, Brian G. Weinshenker, Andrew McKeon, Jan Mendelt Tillema, Vanda A. Lennon, Claudia F. Lucchinetti, Amy Kunchok, Collin M. McClelland, Michael S. Lee, Jeffrey L. Bennett, Victoria S. Pelak, Gregory Van Stavern, Ore Ofe O. Adesina, Eric R. EggenbergerMarie D. Acierno, Dean M. Wingerchuk, Byron L. Lam, Heather Moss, Shannon Beres, Aubrey L. Gilbert, Veeral Shah, Grayson Armstrong, Gena Heidary, Dean M. Cestari, Hadas Stiebel-Kalish, Sean J. Pittock

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

136 Scopus citations


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.MethodsWe 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.ResultsSeventy 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).ConclusionThis 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.

Original languageEnglish
Pages (from-to)E111-E120
Issue number2
StatePublished - 14 Jul 2020


FundersFunder number
Department Laboratory Medicine and Pathology
National Institutes of HealthEY022936
National Institute of Allergy and Infectious DiseasesUM1AI110498
National Institute of Neurological Disorders and StrokePDE10A, R01NS113828
Mayo Clinic
National Multiple Sclerosis SocietyP30 EY 026877, K23 EY 024345
Research to Prevent Blindness


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