Prolonged corticosteroid therapy and steroid-sparing maintenance immunotherapy lower relapse risk in pediatric and adult MOGAD

Abstract

Background

Most existing literature on predictors of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) relapse predates the 2023 diagnostic criteria and have yielded mixed results. While prolonged corticosteroid use has been shown to reduce relapse rates in some studies, the optimal dose and duration remain unclear.

Methods

We conducted a retrospective study of patients treated for MOGAD at the University of Utah and Primary Children's Hospital who met the 2023 diagnostic criteria and either experienced a relapse or were followed for ≥12 months after the initial attack. A multivariable Andersen-Gill model assessed clinical and treatment factors associated with relapse risk. Corticosteroid doses were converted to prednisone equivalents, with duration defined by the period on ≥12.5 mg/day (≥0.16 mg/kg/day in pediatric patients).

Results

Seventy-two patients were included (median onset age 25.5 [IQR 11.75–46.5] years; of these, 47 [65.3 %] female; 30 [41.7 %] pediatric). Twenty-five (34.7 %) experienced at least one relapse. Optic neuritis was more common among adults (81 % vs. 56.7 %, p = 0.049), whereas acute disseminated encephalomyelitis was more frequent among pediatric patients (33.3 % vs. 2.4 %, p < 0.001). Multivariable analysis showed that corticosteroids ≥12 weeks (HR 0.298, 95 % CI 0.1–0.887, p = 0.03) and maintenance immunotherapy (HR 0.061, 95 % CI 0.016–0.229, p < 0.001) were associated with lower relapse risk. Sensitivity analysis showed that both rituximab (HR 0.099, 95 % CI 0.024–0.407, p = 0.001) and maintenance IVIg (HR 0.058, 95 % CI 0.007–0.471, p = 0.008) were independently associated with reduced relapse risk.

Conclusion

Prolonged corticosteroid therapy for ≥12 weeks and maintenance immunotherapy with rituximab or intravenous immunoglobulin independently lower relapse risk in MOGAD.