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Mycophenolate mofetil: An alternative disease-modifying agent for MOG-IgG-associated disorders in childhood: A single-center bidirectional cohort study

Published:August 20, 2022DOI:https://doi.org/10.1016/j.msard.2022.104128

      Highlights

      • Through analysis of thirty pediatric patients diagnosed with MOGAD and treated with MMF for >1 year, our study further identified the efficacy and safety of MMF in treating pediatric MOGAD.
      • We explored risk factors which could predict recurrence during MMF treatment of MOGAD in our study, but no correlation between clinical features and relapse was found.
      • We reported some cases with escalation or de-escalation therapy in MOGAD cohort. Due to the small number of cases, no consistent conclusions can be drawn. Further studies are needed in this respect.

      Abstract

      Objective

      To evaluate the efficacy of mycophenolate mofetil (MMF) in the treatment of childhood MOG-IgG-associated disorder (MOGAD).

      Methods

      Thirty patients diagnosed with relapsing MOGAD and treated with MMF for >1 year from a childhood MOGAD ambispective cohort were included in the study. The clinical characteristics, therapeutic regimen, side effects, annualized relapse rate (ARR), and Expanded Disability Status Scale (EDSS) scores of these patients were evaluated.

      Results

      The median age of disease onset was 7.05 (2.50–12.75) years. The male to female ratio was 1:1.31. All patients used MMF as first-line maintenance treatment. The median time to add MMF from disease onset was 1.08 (0.25−5.00) year. The median number of attacks before MMF initiation was 2 (2 − 8). The median duration of MMF therapy was 2.13 (1.00−3.58) years. Twenty (66.67%) patients did not experience further attacks during MMF therapy. The Kaplan–Meier curves showed a 3-year relapse-free rate of 59.8% (95% CI, 36.62−76.88%). ARR decreased during MMF therapy (0 (0 − 1.72) vs. 1.25 (0.60−4.00); P < 0.05). EDSS stabilized during MMF therapy (1.0 (0 − 2.0) vs. 0 (0 − 2.0); P = 0.206). None of the patients stopped the use of MMF due to intolerable side effects. Onset age, sex, phenotype of the first attack, ARR before MMF, MOG-IgG titers, and combined long-term prednisone (prednisone <10 mg daily for patients >40 kg or <5 mg daily for patients ≤40 kg longer than 6 months) did not predict recurrence during MMF therapy in univariate analysis.

      Conclusions

      MMF was effective and safe for treating childhood MOGAD. No clinical feature that could predict efficacy of MMF was found in pediatric patients with MOGAD.

      Keywords

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