Early Immunotherapy and Longer Corticosteroid Treatment Are Associated With Lower Risk of Relapsing Disease Course in
Margherita Nosadini1, Michael Eyre2, Thea Giacomini2
1From the Paediatric Neurology and Neurophysiology Unit (M.N., I.T., Stefano Sartori), Department of Women's and Children's Health, University Hospital of Padova, Italy; Neuroimmunology Group (M.N., L.Z., Stefano Sartori), Paediatric Research Institute "Città della Speranza," Padova, Italy; School of Biomedical Engineering and Imaging Sciences (M.E.), King's College London, United Kingdom; Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; Unit of Child Neuropsychiatry (Thea Giacomini, R.C., M.M.M.), Clinical and Surgical Neurosciences Department, IRCCS Giannina Gaslini Institute, Genoa, Genoa, Liguria, Italy; Neuroscience Department (M.V., L.P., M.A.N.F.), Bambino Gesù Children's Hospital IRCCS, Rome, Italy; Department of Neurosciences (M.D.C., Antonio Varone), Pediatric Neurology, Santobono-Pausilipon Children's Hospital, Naples, Italy; Unit of Rare Diseases of the Nervous System in Childhood (A.D.P.), Department of Clinical and Experimental Medicine, University of Catania, Italy; Multiple Sclerosis Center (P.A.), ASST della Valle Olona, Hospital of Gallarate, Italy; Child Neurology and Psychiatry Unit (D.M.C., A.F.), Department of Medical and Surgical Sciences (DIMEC), SOrsola Hospital, University of Bologna, Italy; Division of Pediatrics (Giovanni Crichiutti, V.D.), Department of Medicine, University Hospital of Udine, Italy; Unit of Child Neurology and Psychiatry (G.D.R.), Department of Human Pathology of the Adult and Developmental Age "Gaetano Barresi," University of Messina, Italy; Department of Pediatric Neuroscience (E.F., T.G.., N.N., F.R., A.T.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy; GALLO Multiple Sclerosis Centre (P.G., M.M., M.P.), Neurology Clinic, Department of Neuroscience, Università degli Studi di Padova, Italy; Neuroimmunology Laboratory (M.G.), IRCCS Mondino Foundation, Pavia, Italy; Unit of Pediatrics (L.G., C.P.), ULSS 2 Marca Trevigiana, Ca' Foncello Hospital, Treviso, Italy; Institute of Neurology (R.I.), Fondazione Policlinico Universitario "AGemelli" IRCCS, Rome, Italy; Child Neurology Unit and Laboratories (M.L., F.M.), Neuroscience Department, Meyer Children's University Hospital, Florence, Italy; Neurology Unit (Sara Mariotto), Department of Neuroscience, Biomedicine, and Movement Sciences, University of Verona, Policlinico GB Rossi, Italy; Child Neurology and Psychiatry Unit (Sara Matricardi, Sabrina Siliquini), "GSalesi" Children's Hospital, Ospedali Riuniti Ancona, Italy; Child Neuropsychiatry Unit (A.P.), ASST Grande Ospedale Metropolitano Niguarda, Milano; Child Neuropsychiatry Unit (F.P., E.C.T.), Department of Medicine and Surgery, University of Parma, Italy; Pediatric Clinic (Salvatore Savasta, T.F.), Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy; Clinical Neurology (Alberto Vogrig), Azienda Ospedaliero Universitaria Friuli Centrale, Udine, Italy; Department of Neurology (L.Z.), Ospedale San Bortolo, Vicenza, Italy; U.O.CPediatria (S.B., S.R.), Ospedale San Bortolo, Vicenza, Italy; Pediatric Neurology (A.O.), Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy; Child Neuropsychiatry (Gaetano Cantalupo), Department of Surgical Sciences, Dentistry, Gynaecology and Paediatrics, University of Verona, Italy; and Department of Neuroscience (Stefano Sartori), University of Padova, Italy. margherita.nosadini@gmail.com.
Insights
Early immunotherapy and longer corticosteroid treatment reduce relapse risk in pediatric MOGAD. Higher initial disease severity increases the risk of long-term disability in children with myelin oligodendrocyte glycoprotein antibody-associated disorders.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Paediatric-onset myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) require identification of early prognostic factors.
- Understanding factors influencing relapse and long-term outcomes is crucial for managing childhood MOGAD.
Purpose of the Study:
- To identify early clinical and treatment-related factors associated with disease course (relapsing vs. monophasic) in pediatric MOGAD.
- To determine factors predicting final disability (Expanded Disability Status Scale [EDSS] ≥ 1) in pediatric MOGAD patients.
Main Methods:
- Retrospective multicenter cohort study of pediatric MOGAD patients (age ≤ 18 years).
- Comparison of onset features and treatments between monophasic and relapsing disease courses.
- Multivariable logistic regression analysis to identify predictors of relapsing disease and final EDSS ≥ 1.
Main Results:
- Acute disseminated encephalomyelitis was more common in younger children (≤8 years), while optic neuritis predominated in older children (>8 years).
- 40% of patients experienced relapse; early immunotherapy (<7 days), prolonged corticosteroid treatment (≥5 weeks), and abnormal optic nerve MRI at onset were associated with a lower risk of relapse.
- Higher initial EDSS score was independently associated with increased odds of final disability (EDSS ≥ 1).
Conclusions:
- Early and prolonged immunotherapy, along with specific MRI findings, may mitigate relapse risk in pediatric MOGAD.
- Initial disease severity is a key predictor of long-term disability in children with MOGAD.
- These findings aid in risk stratification and management strategies for pediatric MOGAD.
Background And Objectives:
We sought to identify early factors associated with relapse and outcome in paediatric-onset myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD).
Methods:
In a multicenter retrospective cohort of pediatric MOGAD (≤18 years), onset features and treatment were compared in patients with monophasic vs relapsing disease (including cases with follow-up ≥12 months after onset or relapse at any time) and in patients with final Expanded Disability Status Scale (EDSS) 0 vs ≥1 at last follow-up (including cases with follow-up >3 months after last event or EDSS0 at any time). Multivariable logistic regression models were used to evaluate factors associated with relapsing disease course and EDSS ≥ 1 at final follow-up.
Results:
Seventy-five children were included (median onset age 7 years; median 30 months of follow-up). Presentation with acute disseminated encephalomyelitis was more frequent in children aged 8 years or younger (66.7%, 28/42) than in older patients (30.3%, 10/33) (p = 0.002), whereas presentation with optic neuritis was more common in children older than 8 years (57.6%, 19/33) than in younger patients (21.4%, 9/42) (p = 0.001). 40.0% (26/65) of patients relapsed. Time to first relapse was longer in children aged 8 years or younger than in older patients (median 18 vs 4 months) (p = 0.013). Factors at first event independently associated with lower risk of relapsing disease course were immunotherapy <7 days from onset (6.7-fold reduced odds of relapsing course, OR 0.15, 95% CI 0.03-0.61, p = 0.009), corticosteroid treatment for ≥5 weeks (6.7-fold reduced odds of relapse, OR 0.15, 95% CI 0.03-0.80, p = 0.026), and abnormal optic nerves on onset MRI (12.5-fold reduced odds of relapse, OR 0.08, 95% CI 0.01-0.50, p = 0.007). 21.1% (15/71) had EDSS ≥ 1 at final follow-up. Patients with a relapsing course had a higher proportion of final EDSS ≥ 1 (37.5%, 9/24) than children with monophasic disease (12.8%, 5/39) (p = 0.022, univariate analysis). Each 1-point increment in worst EDSS at onset was independently associated with 6.7-fold increased odds of final EDSS ≥ 1 (OR 6.65, 95% CI 1.33-33.26, p = 0.021).
Discussion:
At first attack of pediatric MOGAD, early immunotherapy, longer duration of corticosteroid treatment, and abnormal optic nerves on MRI seem associated with lower risk of relapse, whereas higher disease severity is associated with greater risk of final disability (EDSS ≥ 1).
Related Concept Videos
01:29Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents
01:21Drugs for Treatment of Crohn's Disease in IBD Using Glucocorticoids
01:24Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF
01:25Antiasthma Drugs: Inhaled Corticosteroids and Glucocorticoids
ICS work through a multifaceted mechanism of action. They suppress the inflammatory response caused by the proliferation of TH cells. They also reduce the transcription of the IL-2 gene, which is involved in the...
01:26COPD: Management Using Bronchodilators and Corticosteroids
02:56Treatment Resistant Cancers