- •The most common acute-phase treatments for MOGAD are intravenous corticosteroids and immunoglobulins.
- •The main indication for long-term treatment is relapsing disease course.
- •Corticosteroids, azathioprine, mycophenolate mofetil and rituximab were the most common maintenance treatments.
- •Observational studies have shown less relapses with periodic infusion of intravenous immunoglobulins and rituximab.
Aim to perform a systematic review of the literature on treatment of paediatric patients with MOG-IgG associated disease (MOGAD).
Method We followed the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) statement. The search was conducted in Pubmed (MEDLINE) seeking articles of treatment of MOGAD in patients ≤ 18 years published between January 2012 and April 25th, 2020.
Results We found 72 non-controlled studies (observational studies, case reports and expert recommendations). There were no randomized controlled trials (RCTs). The most commonly reported acute phase treatment was intravenous methylprednisolone in 88% followed by oral steroids in 67%, intravenous human immunoglobulin (IVIG) in 66% and plasma exchange in 33% of the studies. Long-term maintenance treatment was described by 53 studies mainly in relapsing disease course. The most frequently reported treatments were prolonged oral corticosteroids in 53% of the studies followed by azathioprine (51%), mycophenolate mofetil (45%), rituximab (41%) and periodic intravenous immunoglobulin (26%).
Interpretation long-term treatment was reported mainly in relapsing MOGAD paediatric patients. However, the most frequently used medications are not those that have shown higher reduction in the annualised relapse rate in observational studies. RCTs with standardized outcomes are needed to confirm the safety and efficacy of current and new treatments.
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Published online: August 15, 2021
Accepted: August 13, 2021
Received in revised form: July 11, 2021
Received: April 13, 2021
© 2021 Elsevier B.V. All rights reserved.