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

Mathilde Labouret, Stefania Costi, Vincent Bondet, Vincent Trebossen, Enora Le Roux, Alexandra Ntorkou, Sophie Bartoli, Stéphane Auvin, Brigitte Bader-Meunier, Véronique Baudouin, Olivier Corseri, Glory Dingulu, Camille Ducrocq, Cécile Dumaine, Monique Elmaleh, Nicole Fabien, Albert Faye, Isabelle Hau, Véronique Hentgen, Théresa KwonUlrich Meinzer, Naim Ouldali, Cyrielle Parmentier, Marie Pouletty, Florence Renaldo, Isabelle Savioz, Flore Rozenberg, Marie-Louise Frémond, Alice Lepelley, Gillian I Rice, Luis Seabra, Jean-François Benoist, Darragh Duffy, Yanick J Crow, Pierre Ellul, Isabelle Melki

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)615-624
Number of pages10
JournalJournal of clinical immunology
Issue number3
Early online date5 Dec 2022
Publication statusPublished - 1 Apr 2023


  • Humans
  • Child
  • Lupus Vasculitis, Central Nervous System
  • Retrospective Studies
  • Neopterin
  • Neuroinflammatory Diseases
  • Lupus Erythematosus, Systemic/diagnosis
  • Biomarkers


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