Acute Attack Treatment Escalation and Subsequent Relapse Risk in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Abstract

Acute treatment for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) includes high-dose IV methylprednisolone (IVMP), with escalation to IV immunoglobulin (IVIG) and/or plasma exchange (PLEX) based on severity or incomplete response. We evaluated whether escalation at first attack influences disease course. We conducted a retrospective single-center study of pediatric-onset MOGAD evaluated between 2015 and 2024. Patients were classified to (1) steroid-only or (2) steroid-plus (IVMP followed by IVIG, PLEX, or both). Outcomes included early (≤1 year) and overall relapses. Time-to-event analyses used Kaplan-Meier and adjusted Cox regression. Ninety-six of 114 patients were included (58% female; median age 6.3 years [interquartile range (IQR) 4.1-11.3]; median follow-up 3.2 years [IQR 1.4-6.1]). Sixty-eight (71%) received steroid-only therapy and 28 (29%) escalation therapy. Relapses were more frequent with steroid-only treatment at 1 year (28% vs 4%; relative risk [RR] 7.80; 95% CI 1.10-55.3) and at final follow-up (43% vs 11%; RR 3.98; 95% CI 1.33-11.93). Escalation therapy was associated with reduced relapse risk (adjusted hazard ratio [HR] 0.25; 95% CI 0.07-0.85; <i>p</i> = 0.026; log-rank <i>p</i> = 0.006), consistent across propensity score-adjusted analyses (HR 0.24; 95% CI 0.07-0.81; <i>p</i> = 0.022). Acute immunotherapy escalation was associated with lower early and mid-term relapses, suggesting the first attack may represent a therapeutic opportunity to influence disease trajectory.