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

Abstract

IMPORTANCE 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.OBJECTIVE To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD. DESIGN, SETTING, AND PARTICIPANTSThis 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. EXPOSURES Maintenance IVIG.MAIN OUTCOMES AND MEASURES Relapse rates while receiving maintenance IVIG compared with before initiation of therapy. RESULTSOf 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) (t 108 = 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.CONCLUSIONS AND RELEVANCE 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.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management

“…The risk of relapse was lower in those treated with higher IVIG doses or more frequently (e.g., 0.4 g/kg every week, or 2 g/kg every 4 weeks). Two patients (3%) experienced worsening disability (EDSS score) during IVIG treatment (167). The high costs and restricted availability are the main elements that limit the use of this treatment in a larger scale.…”

Effectiveness and tolerability of different therapies in preventive treatment of MOG-IgG-associated disorder: A network meta-analysis

“…The use of IVIG is more common in other CNS demyelinating diseases, such as MS, as compared to MOG-AD. Olyaeemanesh et al ( 21) performed a meta-analysis including six randomized (24) showed that maintenance IVIG was effective in reducing relapses. To date, IVIG has not been considered as a firstor second-line therapy for MOG-AD due to insufficient clinical evidence.…”

“…large sample sizes and a lack of comparative groups were not included, for example, Ringelstein et al (33) found blocking IL-6R was safe and effective in treating MOG-AD; Chen et al and Hacohen et al (24) recently performed a multicenter cohort study and confirmed the efficacy of IVIG in treating MOG-AD; therefore, the exclusion of single-arm studies may exaggerate or underestimate the efficacy of certain treatments. Third, the definite therapeutic efficacy could not be assessed due to the combination of immunosuppressors with OC in some studies, and many of the studies have included patients more than once if they have been on multiple treatments.…”

“…For MOG-AD, a recent multicenter study by Chen et al. ( 24 ) showed that maintenance IVIG was effective in reducing relapses. To date, IVIG has not been considered as a first- or second-line therapy for MOG-AD due to insufficient clinical evidence.…”

Neuro-ophthalmological Presentation of Optic Neuritis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

“…Long-term treatment is recommended for patients at risk for relapse and current therapies comprise the off-label use of prednisolone, steroid-sparing immunosuppression with azathioprine, methotrexate, mycophenolate mofetil, rituximab and IVIG [75][76][77][78][79]. Maintenance treatment is given either as monotherapy or as combination therapy [9,38,42].…”