Treatment Response In Children With Relapsing Mogad

Background Patients with MOG-associated disease are thought to have a favorable outcome, but reports of relapsing disease course have emerged. Data regarding treatment in relapsing MOGAD are lacking. Our purpose was to evaluate treatment response of intravenous immunoglobulins (IVIG) compared to other therapies in children with relapsing MOGAD. Methods Pediatric patients with MOGAD were recruited from our ongoing BIOMARKER-study and different international medical centers. Inclusion criteria encompassed: age <18 years, diagnosis of MOGAD based on recently suggested diagnostic criteria, clinical presentation with relapsing disease course, detailed treatment history of more than 24 months. Results 119 children were included with a median age of 6 years. Patients presented with ON (n=24), ADEM (n=62), NMOSD (n=27), cortical encephalitis (n=4), myelitis (n=2). Patients received IVIG (n=18), IVIG plus prednisone (n=7), other immunotherapy (n=37; e.g. mycophenolate mofetil, Azathioprine, Rituximab, first-line MS therapies, steroids), IVIG plus other therapies (n=31), 26 patients received no treatment. Annual relapse rate (ARR) after start of treatment was lower in all treatment groups compared to no treatment (p<0.001). 39% of children with isolated IVIG suffered from relapses, compared to 62% with other therapies, 86% in the IVIG plus prednisone, 87% in the IVIG plus DMT and 100% in untreated group (p<0.001). Patients with only IVIG had the lowest ARR (median 0.00, range: 0.00-0.27). Separate comparison of therapies showed lowest number of relapses in patients with IVIG. Conclusion Our study indicates that IVIG has a favorable treatment effect in relapsing MOGAD with the lowest ARR.