PT  - JOURNAL ARTICLE
AU  - Chen, John J.
AU  - Flanagan, Eoin P.
AU  - Bhatti, M. Tariq
AU  - Jitprapaikulsan, Jiraporn
AU  - Dubey, Divyanshu
AU  - Lopez Chiriboga, Alfonso (Sebastian) S.
AU  - Fryer, James P.
AU  - Weinshenker, Brian G.
AU  - McKeon, Andrew
AU  - Tillema, Jan-Mendelt
AU  - Lennon, Vanda A.
AU  - Lucchinetti, Claudia F.
AU  - Kunchok, Amy
AU  - McClelland, Collin M.
AU  - Lee, Michael S.
AU  - Bennett, Jeffrey L.
AU  - Pelak, Victoria S.
AU  - Van Stavern, Gregory
AU  - Adesina, Ore-Ofe O.
AU  - Eggenberger, Eric R.
AU  - Acierno, Marie D.
AU  - Wingerchuk, Dean M.
AU  - Lam, Byron L.
AU  - Moss, Heather
AU  - Beres, Shannon
AU  - Gilbert, Aubrey L.
AU  - Shah, Veeral
AU  - Armstrong, Grayson
AU  - Heidary, Gena
AU  - Cestari, Dean M.
AU  - Stiebel-Kalish, Hadas
AU  - Pittock, Sean J.
TI  - Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder
AID  - 10.1212/WNL.0000000000009758
DP  - 2020 Jul 14
TA  - Neurology
PG  - e111--e120
VI  - 95
IP  - 2
4099  - http://n.neurology.org/content/95/2/e111.short
4100  - http://n.neurology.org/content/95/2/e111.full
SO  - Neurology2020 Jul 14; 95
AB  - 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.ADEM=acute disseminating encephalomyelitis; AQP4=aquaporin-4; ARR=annualized relapse rate; IgG=immunoglobulin G; IVIG=IV immunoglobulin; MOG=myelin oligodendrocyte glycoprotein; MOGAD=MOG-IgG associated disorder; MS=multiple sclerosis; NMOSD=neuromyelitis optica spectrum disorder