Association of Maintenance Intravenous Immunoglobulin With Prevention of Relapse in Adult Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease

• DOI: 10.1001/jamaneurol.2022.0489
• Publication Date: 2022-04-04T15:03:29Z
• Authors: John J. Chen; Saif Huda; Yael Hacohen; Michael Levy; Itay Lotan; Adi Wilf-Yarkoni; Hadas Stiebel-Kalish; Mark A. Hellmann; Elias S. Sotirchos; Amanda D. Henderson; Sean J. Pittock; M. Tariq Bhatti; Eric R. Eggenberger; Marie Di Nome; Ho Jin Kim; Su-Hyun Kim; Albert Saiz; Friedemann Paul; Russell C. Dale; Sudarshini Ramana...; Jacqueline Palace; Valentina Camera; Maria Isabel Leite; Byron L. Lam; Jeffrey L. Bennett; Sara Mariotto; Dave Hodge; Bertrand Audoin; Elisabeth Maillart; Romain Deschamps; Julie Pique; Eoin P. Flanagan; Romain Marignier
• Keywords: Male; maintenance intravenous immunoglobulin (IVIG); Immunoglobulins; Immunoglobulins, Intravenous; adult patients; 3. Good health; Cohort Studies; 03 medical and health sciences; 0302 clinical medicine; reduction in relapse frequency; Recurrence; Chronic Disease; Humans; Immunologic Factors; Female; Myelin-Oligodendrocyte Glycoprotein; Intravenous; Child; myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD); Autoantibodies; Retrospective Studies

Recent studies suggest that maintenance intravenous immunoglobulin (IVIG) may be an effective treatment to prevent relapses in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD); however, most of these studies had pediatric cohorts, and few studies have evaluated IVIG in adult patients.To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD.This was a retrospective cohort study conducted from January 1, 2010, to October 31, 2021. Patients were recruited from 14 hospitals in 9 countries and were included in the analysis if they (1) had a history of 1 or more central nervous system demyelinating attacks consistent with MOGAD, (2) had MOG-IgG seropositivity tested by cell-based assay, and (3) were age 18 years or older when starting IVIG treatment. These patients were retrospectively evaluated for a history of maintenance IVIG treatment.Maintenance IVIG.Relapse rates while receiving maintenance IVIG compared with before initiation of therapy.Of the 876 adult patients initially identified with MOGAD, 59 (median [range] age, 36 [18-69] years; 33 women [56%]) were treated with maintenance IVIG. IVIG was initiated as first-line immunotherapy in 15 patients (25%) and as second-line therapy in 37 patients (63%) owing to failure of prior immunotherapy and in 7 patients (12%) owing to intolerance to prior immunotherapy. The median (range) annualized relapse rate before IVIG treatment was 1.4 (0-6.1), compared with a median (range) annualized relapse rate while receiving IVIG of 0 (0-3) (t108 = 7.14; P < .001). Twenty patients (34%) had at least 1 relapse while receiving IVIG with a median (range) time to first relapse of 1 (0.03-4.8) years, and 17 patients (29%) were treated with concomitant maintenance immunotherapy. Only 5 of 29 patients (17%) who received 1 g/kg of IVIG every 4 weeks or more experienced disease relapse compared with 15 of 30 patients (50%) treated with lower or less frequent dosing (hazard ratio, 3.31; 95% CI, 1.19-9.09; P = .02). At final follow-up, 52 patients (88%) were still receiving maintenance IVIG with a median (range) duration of 1.7 (0.5-9.9) years of therapy. Seven of 59 patients (12%) discontinued IVIG therapy: 4 (57%) for inefficacy, 2 (29%) for adverse effects, and 1 (14%) for a trial not receiving therapy after a period of disease inactivity.Results of this retrospective, multicenter, cohort study of adult patients with MOGAD suggest that maintenance IVIG was associated with a reduction in disease relapse. Less frequent and lower dosing of IVIG may be associated with treatment failure. Future prospective randomized clinical trials are warranted to confirm these findings.