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

Research output: Contribution to journalArticlepeer-review

12 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 (US)
Pages (from-to)E111-E120
Issue number2
StatePublished - Jul 14 2020

Bibliographical note

Funding Information:
This work was supported by the Department Laboratory Medicine and Pathology and the Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, MN. This work was also supported by the Leonard and Mary Lou Hoeft Career Development Award in Ophthalmology Research. J.L. Bennett was supported by grants from the NIH (EY022936, UM1AI110498) and the National Multiple Sclerosis Society. H.E. Moss was supported by grants from the NIH (K23 EY 024345, P30 EY 026877) and Research to Prevent Blindness (unrestricted grant to Stanford University Department of Ophthalmology).

Funding Information:
J.J. Chen reports no disclosures. E.P. Flanagan was a site principal investigator in a randomized placebo-controlled clinical trial of inebilizumab (a CD19 inhibitor) in NMOSDs funded by MedImmune/Viela Bio and is funded by NIH National Institute of Neurological Disorders and Stroke (R01NS113828). M.T. Bhatti and J. Jitprapaikulsan report no disclosures. D. Dubey has received research support from Center of Multiple Sclerosis and Autoimmune Neurology, Center for Clinical and Translational Science, and Grifols Pharmaceuticals. He has consulted for UCB pharmaceuticals. All compensation for consulting activities is paid directly to Mayo Clinic. Dr. Dubey has a patent pending for Kelch-like protein 11 as a marker of neurologic autoimmunity. A.S. Lopez Chiriboga and J.P. Fryer report no disclosures. B.G. Weinshenker receives royalties from RSR Ltd, Oxford University, Hospices Civil de Lyon, and MVZ Labor PD. A. McKeon has received research funding from Alexion, Grifols, and Medimmune and has patents pending for the following IgGs as biomarkers of autoimmune neurologic disorders: septin-5, Kelch-like protein 11, GFAP, PDE10A, and MAP1B. J.M. Tillema reports no disclosures. V.A. Lennon receives royalties for technology relating to AQP4 antibodies for diagnosis of neuromyelitis optica (NMO) and its spectrum disorders and is a named inventor on filed patents that relate to functional AQP4/NMO-IgG assays and NMO-IgG as a cancer marker. C.F. Lucchinetti is a named inventor on filed patents that relate to NMO-IgG and has received research funding from Biogen, Novartis, Alexion, Mallinkrodt, and the National MS Society. A. Kunchok and C.M. McClelland report no disclosures. M.S. Lee is a named inventor on filed patents on potentiating botulinum toxin and received research support from Quark. J.L. Bennett serves as a consultant for VielaBio, EMD Serono, Alexion, Chugai, Genentech, Roche, and Clene Neuroscience; receives license and royalties for a patent on compositions and methods for the treatment of neuromyelitis optica (aquaporumab); and receives research support from Mallinckrodt Pharmaceuticals. V.S. Pelak receives royalties from Up-to-Date. G. Van Stavern, O.O.

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