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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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To identify central nervous system (CNS) disease biomarkers of j-NPSLE.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>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-\u03b1) protein levels in cerebrospinal fluid (CSF) were assessed, together with routine biological and radiological investigations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      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 (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0008). Neopterin and IFN-\u03b1 protein levels in CSF were significantly higher at diagnosis of j-NPSLE with CNS involvement than after resolution of NP features (respectively\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0015 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0010) upon immunosuppressive treatment in all patients tested (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200910). Both biomarkers correlated strongly with each other (\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sub>\n                        <jats:italic>s<\/jats:italic>\n                      <\/jats:sub>\n                      \u2009=\u20090.832,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001,\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200923 paired samples).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CSF IFN-\u03b1 and neopterin constitute promising biomarkers useful in the diagnosis and monitoring of activity in j-NPSLE.<\/jats:p>\n                  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The data processing has been approved by AP-HP (Public Hospitals of Paris) data protection office and registered in general data processing register (N\u00b020200227113056). Parents or patients, if they had reached the age of majority by the time of the study, have been informed in writing. 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