Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers - Institut Pasteur Access content directly
Journal Articles Journal of Clinical Immunology Year : 2022

Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers

1 AP-HP Hôpital universitaire Robert-Debré [Paris]
2 RAISE - Centre de référence des rhumatismes inflammatoires et maladies autoimmunes systémiques rares de l'enfant [Paris]
3 SU - Sorbonne Université
4 UNIMI - Università degli Studi di Milano = University of Milan
5 Immunologie Translationnelle - Translational Immunology lab
6 UFR Médecine UPCité - UFR Médecine [Santé] - Université Paris Cité
7 CIC 1426 - Centre d'Investigation Clinique 1426
8 ECEVE (U1123 / UMR_S_1123) - Epidémiologie Clinique et Evaluation Economique Appliquées aux Populations Vulnérables
9 NeuroDiderot (UMR_S_1141 / U1141) - Maladies neurodéveloppementales et neurovasculaires
10 IUF - Institut universitaire de France
11 Hôpital Necker - Enfants Malades [AP-HP]
12 Equipe Inserm U1163 - Immunogenetics of pediatric autoimmune diseases
13 Centre Hospitalier Sud Francilien
14 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
15 CHI Créteil
16 CHV - Centre Hospitalier de Versailles André Mignot
17 CRI (UMR_S_1149 / ERL_8252 / U1149) - Centre de recherche sur l'Inflammation
18 Biologie et Génétique de la Paroi bactérienne - Biology and Genetics of Bacterial Cell Wall
19 CHU Trousseau [APHP]
20 GHU AP-HP Centre Université Paris Cité
21 Equipe Inserm U1163 - Laboratory of neurogenetics and neuroinflammation
22 University of Manchester [Manchester]
23 Université Paris-Saclay
24 Edin. - University of Edinburgh
25 I3 - Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière]
26 CHU Bordeaux
Isabelle Hau
  • Function : Author

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 ( R s = 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.
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pasteur-03889611 , version 1 (08-12-2022)

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Mathilde Labouret, Stefania Costi, Vincent Bondet, Vincent Trebossen, Enora Le Roux, et al.. Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers. Journal of Clinical Immunology, 2022, ⟨10.1007/s10875-022-01407-1⟩. ⟨pasteur-03889611⟩
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