Predictors of a Relapsing Course in Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease
Akash Virupakshaiah1, Vinicius Schoeps1, Jonathan Race2, Michael Waltz2, Siefaddeen Sharayahn3, Zahra Nasr1, Carson Moseley1, Scott Zamvil1, Cristina Gaudioso3, Allison Schuette2, Charles Casper2, John Rose2, Eoin Flanagan4, Moses Rodriguez4, Jan-Mendelt Tillema4, Tanuja Chitnis5, Mark Gorman6, Jennifer Graves7, Leslie Benson6, Mary Rensel8, Aaron Abrams8, Lauren Krupp9, Timothy Lotze10, Gregory Aaen11, Yolanda Wheeler12, Teri Schreiner13, Amy Waldman14, Janet Chong1, Soe Mar3, Emmanuelle Waubant1
1Neurology, UCSF, 2University of Utah, 3Neurology, Washington University School of Medicine in St. Louis, 4Mayo Clinic, 5Brigham and Women's Hospital, 6Neurology, Boston Children's Hospital, 7UCSD, 8Cleveland Clinic, 9NYU Langone Medical Center, 10Texas Children's Hospital, 11Loma Linda University School of Medicine, 12University of Alabama at Birmingham, 13University of Colorado/ Children's Hospital of Colorado, 14Children's Hospital of Philadelphia
Objective:
The study aimed to identify factors at MOGAD onset that are associated with a multiphasic course.
Background:
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently identified demyelinating disorder, and children represent about 50% of all cases. Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management.
Design/Methods:
Prospectively collected data from pediatric patients with MOGAD seen by the US Network of Pediatric MS Centers were leveraged. Univariable and multivariable models were used to predict recurrent disease after adjusting for confounders.
Results:
We identified 326 MOGAD cases (mean age at first event 8.9 years (SD 4.3), 57% female, 77% White, 74% non-Hispanic), and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.64, 95% CI 1.16-2.33, p=0.005) and Hispanic/Latino ethnicity (HR 1.65, 95% CI 1.11-2.45, p=0.012) were associated with a higher risk of relapsing MOGAD. Non-Whites had a higher estimated risk of relapse (HR 1.47, 95% CI 0.98-2.20, p=0.063). Maintenance treatment initiated before a second event with an anti-CD20 agent (HR 0.25, 95% CI 0.07-0.92, p=0.037) or intravenous immunoglobulin (IVIG) (HR 0.35, 95% CI 0.14-0.88, p=0.026) was associated with lower risk of a second event in multivariable analyses. Conversely, maintenance steroids were associated with a higher estimated relapse risk (HR 1.76, 95% CI 0.90-3.45, p=0.097). Other demographic, clinical, biological, and MRI characteristics at onset did not show significant associations with the risk of having a relapse.
Conclusions:
Sex and ethnicity, and potentially race are associated with relapsing MOGAD. Use of anti-CD20 or IVIG therapy shortly after onset is associated with a lower risk of second event. Preventive treatment after a first event could be considered in those with a higher relapse risk.