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Treatment of MOG-IgG associated disease in paediatric patients: A systematic review
Bruna Klein da Costa1, Brenda Louise Banwell2, Douglas Kazutoshi Sato1
1Neurology Department, School of Medicine, Pontifical Catholic University of Rio Grande do Sul (PUCRS), Brazil / Brain Institute of Rio Grande do Sul, Av. Ipiranga, 6690, Porto Alegre 90610-000, Brazil.
Abstract:
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.
Insights
This systematic review found no randomized controlled trials for MOG-IgG associated disease (MOGAD) in children. Commonly used treatments may not be the most effective for reducing relapses, highlighting the need for further research.
Area of Science:
- Pediatric Neurology
- Immunology
- Systematic Review
Background:
- Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disease (MOGAD) is a rare autoimmune demyelinating disorder.
- Optimal treatment strategies for pediatric MOGAD remain unclear due to a lack of high-quality evidence.
Purpose of the Study:
- To systematically review the existing literature on the treatment of MOG-IgG associated disease in pediatric patients.
- To identify commonly used acute and maintenance therapies and assess their reported efficacy.
Main Methods:
- A systematic literature search was conducted using PubMed (MEDLINE) for studies published between January 2012 and April 2020.
- Included studies focused on MOGAD treatment in patients aged 18 years or younger.
- Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were followed.
Main Results:
- The review identified 72 non-controlled studies; no randomized controlled trials (RCTs) were found.
- Intravenous methylprednisolone was the most common acute treatment (88%).
- For maintenance, prolonged oral corticosteroids (53%), azathioprine (51%), and mycophenolate mofetil (45%) were frequently reported, particularly for relapsing MOGAD.
Conclusions:
- Current treatment approaches for pediatric MOGAD, especially in relapsing cases, are primarily based on observational data and expert opinion.
- Frequently used treatments did not consistently demonstrate significant reductions in annualized relapse rates in observational studies.
- There is a critical need for well-designed RCTs with standardized outcomes to establish the safety and efficacy of MOGAD treatments in children.
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