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Rarity of silent progression: PIRA and RAW in myelin oligodendrocyte glycoprotein antibody-associated disease
Ki Hoon Kim1, You-Ri Kang2, Su-Hyun Kim3
1Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Abstract:
In myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), disability is typically attack-related and frequently remain stable over time if no relapses occur. This retrospective cohort study assessed the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in Korean patients with MOGAD from five referral centers. Among 205 patients, 116 with ⩾1 year of follow-up and a minimum of 3 documented EDSS scores were eligible for inclusion. Over a mean follow-up of 62.2 months, PIRA occurred in two patients (1.7%) and RAW in six patients (5.2%), highlighting that PIRA is a rare and atypical event in MOGAD.
Insights
Progression independent of relapse activity (PIRA) is rare in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). This study found PIRA in only 1.7% of Korean MOGAD patients, with relapse-associated worsening being more common.
Area of Science:
- Neurology
- Immunology
- Neuroimmunology
Background:
- Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a demyelinating condition where disability is typically linked to attacks.
- The long-term disability progression patterns, specifically progression independent of relapse activity (PIRA), are not well-characterized in MOGAD.
Purpose of the Study:
- To determine the prevalence of PIRA and relapse-associated worsening (RAW) in a cohort of Korean patients with MOGAD.
- To understand the long-term disability accrual in MOGAD.
Main Methods:
- Retrospective cohort study involving 205 Korean patients with MOGAD from five referral centers.
- Included 116 patients with at least one year of follow-up and a minimum of three documented Expanded Disability Status Scale (EDSS) scores.
- Assessed PIRA and RAW over a mean follow-up of 62.2 months.
Main Results:
- Progression independent of relapse activity (PIRA) was observed in only two patients (1.7%).
- Relapse-associated worsening (RAW) occurred in six patients (5.2%).
- PIRA is a rare and atypical event in MOGAD.
Conclusions:
- PIRA is uncommon in Korean patients with MOGAD.
- Disability progression in MOGAD is predominantly associated with relapses.
- Further research is needed to fully understand the long-term trajectory of MOGAD.
