Neuropediatrics 2023; 54(S 01): S1-S32
DOI: 10.1055/s-0043-1777154
Neuroimmunologie

Treatment Response in Children with Relapsing MOGAD

Authors

  • E.-M. Wendel

    1   Neuropädiatrie, Olgahospital/Klinikum Stuttgart, Stuttgart, Deutschland

  • A. Bertolini

    2   Neuropädiatrie, Vestische Kinder- und Jugendklinik, Datteln, Deutschland

  • A. Blaschek

    3   Neuropädiatrie, Haunersches Kinderspital, LMU München, München, Deutschland

  • F. Brilot

    4   The Kids Research Institute at the Children's Hospital at Westmead, Sydney Medical School, University of Sydney, Brain Autoimmunity Group, Sydney, Australien

  • J. J. Chen

    5   Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, Vereinigte Staaten von Amerika

  • R. Dale

    4   The Kids Research Institute at the Children's Hospital at Westmead, Sydney Medical School, University of Sydney, Brain Autoimmunity Group, Sydney, Australien

  • K. Deiva

    6   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Hôpital Bicêtre, Paris, Frankreich

  • T. Foiadelli

    7   Clinica Pediatrica, Fondazione IRCCS Policlinico San Matteo, Università degli Studi di Pavia, Pavia, Italien

  • L. Giorgi

    6   Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Hôpital Bicêtre, Paris, Frankreich

  • S. Huda

    8   Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, Vereinigtes Königreich

  • M. Karenfort

    9   Neuropädiatrie, Universitätsklinikum Düsseldorf, Düsseldorf, Deutschland

  • S. Mariotto

    10   Neurology Unit, Department of Neurosciences, Biomedicine, and Movement Sciences, University of Verona, Verona, Italien

  • S. Ramanathan

    11   Translational Neuroimmunology Group, Kids Neuroscience Centre, Department of Neurology, Concord Hospital, Children's Hospital at Westmead, Sydney, Australien

  • M. Schimmel

    12   Neuropädiatrie, Medizinische Universität Augsburg, Augsburg, Deutschland

  • V. Shah

    13   Division of Pediatric Ophthalmology, Cincinnati Children's Hospital, Cincinnati, Vereinigte Staaten von Amerika

  • C. Thiels

    14   Neuropädiatrie, Klinik für Kinder- und Jugendmedizin der Ruhr-Universität Bochum, Bochum, Deutschland

  • K. Schanda

    15   Neurologie, Medizinische Universität Innsbruck, Innsbruck, Österreich

  • M. Reindl

    15   Neurologie, Medizinische Universität Innsbruck, Innsbruck, Österreich

  • K. Rostasy

    2   Neuropädiatrie, Vestische Kinder- und Jugendklinik, Datteln, Deutschland

  • E.-M. Wendel

    1   Neuropädiatrie, Olgahospital/Klinikum Stuttgart, Stuttgart, Deutschland
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Background: Patients with MOG-associated disease (MOGAD) are thought to have a favorable outcome, but reports of relapsing disease course with poor outcome have recently emerged. Sufficient data regarding treatment in children with relapsing MOGAD are lacking. Our purpose was to evaluate the treatment response of intravenous immunoglobulins (IVIG) compared with other therapies in children with relapsing disease course.

Methods: Pediatric patients with MOGAD were recruited from our ongoing BIOMARKER study and different international medical centers. Inclusion criteria encompassed: age <18 years, diagnosis of MOGAD based on recently suggested diagnostic criteria (Barnwell, 2023), clinical presentation with relapsing disease course, detailed treatment history of more than 24 months.

Results: A total of 119 children were included with a median age of 6 years. Patients presented with ON (n = 24), ADEM (n = 62), NMOSD (n = 27), cortical encephalitis (n = 4), myelitis (n = 2). Patients received IVIG (n = 18), IVIG plus prednisone (n = 7), other immunotherapy (n = 37; e.g., mycophenolate mofetil, azathioprine, rituximab, first-line MS therapies, steroids), IVIG plus other therapies (n = 31), 26 patients received no treatment. Annual relapse rate (ARR) after start of treatment was lower in all treatment groups compared with no treatment (p < 0.001). 39% of children with isolated IVIG suffered from relapses, compared with 62% with other therapies, 86% in the IVIG plus prednisone, 87% in the IVIG plus DMT, and 100% in the untreated group (p < 0.001). Patients with only IVIG had the lowest ARR (median: 0.00, range: 0.00–0.27). Separate comparison of therapies (IVIG, RTX, AZA, MMF, Predni, GLAT, INFß, NTZ, chemotherapeutics, IVIG plus other therapy) showed lowest number of relapses in patients with IVIG.

Conclusion: Our study indicates that IVIG has a favorable treatment effect in relapsing MOGAD with the lowest ARR. Prospective studies are warranted to identify the optimal treatment.



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Artikel online veröffentlicht:
13. November 2023

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