학술논문
Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers.
Document Type
Article
Author
Labouret, Mathilde; Costi, Stefania; Bondet, Vincent; Trebossen, Vincent; Le Roux, Enora; Ntorkou, Alexandra; Bartoli, Sophie; Auvin, Stéphane; Bader-Meunier, Brigitte; Baudouin, Véronique; Corseri, Olivier; Dingulu, Glory; Ducrocq, Camille; Dumaine, Cécile; Elmaleh, Monique; Fabien, Nicole; Faye, Albert; Hau, Isabelle; Hentgen, Véronique; Kwon, Théresa
Source
Subject
*SYSTEMIC lupus erythematosus
*AUTOIMMUNE diseases
*CENTRAL nervous system
*MILD cognitive impairment
*NEUROINFLAMMATION
*NEOPTERIN
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Language
ISSN
0271-9142
Abstract
Introduction: Juvenile systemic lupus erythematosus (j-SLE) is a rare chronic autoimmune disease affecting multiple organs. Ranging from minor features, such as headache or mild cognitive impairment, to serious and life-threatening presentations, j-neuropsychiatric SLE (j-NPSLE) is a therapeutic challenge. Thus, the diagnosis of NPSLE remains difficult, especially in pediatrics, with no specific biomarker of the disease yet validated. Objectives: To identify central nervous system (CNS) disease biomarkers of j-NPSLE. Methods: A 5-year retrospective tertiary reference monocentric j-SLE study. A combination of standardized diagnostic criteria and multidisciplinary pediatric clinical expertise was combined to attribute NP involvement in the context of j-SLE. Neopterin and interferon-alpha (IFN-α) protein levels in cerebrospinal fluid (CSF) were assessed, together with routine biological and radiological investigations. Results: Among 51 patients with j-SLE included, 39% presented with j-NPSLE. J-NPSLE was diagnosed at onset of j-SLE in 65% of patients. No specific routine biological or radiological marker of j-NPSLE was identified. However, CSF neopterin levels were significantly higher in active j-NPSLE with CNS involvement than in j-SLE alone (p = 0.0008). Neopterin and IFN-α protein levels in CSF were significantly higher at diagnosis of j-NPSLE with CNS involvement than after resolution of NP features (respectively p = 0.0015 and p = 0.0010) upon immunosuppressive treatment in all patients tested (n = 10). Both biomarkers correlated strongly with each other (Rs = 0.832, p < 0.0001, n = 23 paired samples). Conclusion: CSF IFN-α and neopterin constitute promising biomarkers useful in the diagnosis and monitoring of activity in j-NPSLE. [ABSTRACT FROM AUTHOR]