Diagnostic and Therapeutic Insights Into Pediatric Neurosarcoidosis: Observations From French Pediatric Rheumatology Centers.

Autor: Tohme R; Department of General Pediatrics, Pediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Pediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Tanase A; Department of Pediatric Radiology, Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Dumaine C; Department of General Pediatrics, Pediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Pediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Dusser P; Department of Pediatric Rheumatology and CEREMAIA, National Reference Centre for Autoinflammatory Diseases and Inflammatory Amyloidosis, Bicêtre University Hospital, APHP, and University of Paris Saclay, Le Kremlin-Bicêtre, France., Adle-Biassette H; Department of Pathology, Lariboisière Hospital, DMU DREAM, APHP, Inserm, NeuroDiderot Université Paris Cité, Paris, France., Despert V; Department of Pediatric, Rennes University Hospital, Rennes, France., Faye A; Department of General Pediatrics, Pediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Pediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Mannes I; Department of Pediatric Radiology, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France., Melki I; Department of General Pediatrics, Pediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Pediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Kone-Paut I; Department of Pediatric Rheumatology and CEREMAIA, National Reference Centre for Autoinflammatory Diseases and Inflammatory Amyloidosis, Bicêtre University Hospital, APHP, and University of Paris Saclay, Le Kremlin-Bicêtre, France., Meinzer U; Department of General Pediatrics, Pediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Pediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Cité, INSERM, Centre de Recherche sur l'inflammation UMR 1149, Paris, France. Electronic address: ulrich.meinzer@aphp.fr.
Jazyk: angličtina
Zdroj: Pediatric neurology [Pediatr Neurol] 2024 Oct 18; Vol. 162, pp. 12-20. Date of Electronic Publication: 2024 Oct 18.
DOI: 10.1016/j.pediatrneurol.2024.10.007
Abstrakt: Background: The diagnosis and management of neurosarcoidosis (NS) in pediatric patients remain challenging, with limited case documentation to guide clinicians. Most existing reports focus on initial presentations. This study aimed to outline the clinical features, management, and medium-term outcomes of pediatric NS METHODS: In this retrospective, multicentric, observational study, we collected data from pediatric patients followed in French pediatric rheumatology centers with a diagnosis of NS between January 2001 and June 2023.
Results: We identified 11 patients diagnosed with NS, comprising eight girls and three boys. The mean age at diagnosis of sarcoidosis was 10 (5 to 15) years, and the mean age of diagnosis of NS was 11.5 (5 to 17) years. Predominant neurological symptoms included headache (nine of 11 patients), papilledema (6 of 11 patients), facial palsy (two patients), seizures (one patient), and motor deficit (two patients). Nine of 11 patients had eye involvement, which consisted of granulomatous and bilateral uveitis. All patients exhibited meningitis, with cerebrospinal fluid white blood cell counts ranging from 6 to 70 cells/mm 3 . Six individuals presented neurological abnormalities on imaging, detailed in this study. Treatment primarily involved corticosteroids, methotrexate, and tumor necrosis factor alpha (TNF-alpha) inhibitors. Biologics targeting TNF-alpha were necessary to achieve remission in eight of 11 patients. In two patients who did not receive this treatment initially, it was required later in the course of evolution.
Conclusions: This study enhances understanding of the clinical course of pediatric NS and supports the early use of TNF-alpha biologics for improved management in affected children.
Competing Interests: Declaration of competing interest We declare no competing interest.
(Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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