4.7 Article

Relapse activity in the chronic phase of anti-myelin-oligodendrocyte glycoprotein antibody-associated disease

Journal

JOURNAL OF NEUROLOGY
Volume 269, Issue 6, Pages 3136-3146

Publisher

SPRINGER HEIDELBERG
DOI: 10.1007/s00415-021-10914-x

Keywords

Anti-myelin oligodendrocyte glycoprotein (MOG) antibody; MOG-antibody-associated disease (MOGAD); Neuromyelitis optica spectrum disorder (NMOSD); Relapse-free survival; Relapse prevention

Funding

  1. MHLW Program [20FC1030]
  2. JSPS KAKENHI [20K07892]
  3. Grants-in-Aid for Scientific Research [20K07892] Funding Source: KAKEN

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Patients with MOGAD have a lower risk of relapse compared to those with AQP4-Ab-positive NMOSD, especially 5 years after onset. However, relapses later than 10 years from onset are not rare in both diseases.
Objective The patterns of relapse and relapse-prevention strategies for anti-myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are not completely investigated. We compared the patterns of relapse in later stages of MOGAD with those of anti-aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD). Methods In this observational, comparative cohort study, 66 patients with MOGAD and 90 with AQP4-Ab-positive NMOSD were enrolled. We compared the patterns of relapse and annualized relapse rates (ARRs) in the first 10 years from disease onset, stratified by relapse-prevention treatments. Results Approximately 50% of the patients with MOGAD experienced relapses in the first 10 years. Among those not undergoing relapse-prevention treatments, ARRs in the first 5 years were slightly lower in MOGAD patients than in AQP4-Ab-positive NMOSD patients (MOGAD vs. AQP4-Ab NMOSD: 0.19 vs. 0.30; p = 0.0753). After 5 years, the ARR decreased in MOGAD patients (MOGAD vs. AQP4-Ab NMOSD: 0.05 vs. 0.34; p = 0.0001), with a 72% reduction from the first 5 years (p = 0.0090). Eight (61.5%) of the 13 MOGAD patients with more than 10-year follow-up from disease onset showed relapse 10 years after onset. Clustering in the timing and phenotype of attacks was observed in both disease patients. The effectiveness of long-term low-dose oral PSL for relapse prevention in patients with MOGAD has not been determined. Conclusions The relapse risk in patients with MOGAD is generally lower than that in patients with AQP4-Ab-positive NMOSD, especially 5 years after onset. Meanwhile, relapses later than 10 years from onset are not rare in both diseases.

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