作者
Mathilde Labouret,Stefania Costi,Vincent Bondet,Vincent Trebossen,Énora Le Roux,Alexandra Ntorkou,Sophie Bartoli,Stéphane Auvin,Brigitte Bader‐Meunier,Véronique Baudouin,Olivier Corseri,Glory Dingulu,Charles Ducrocq,Cécile Dumaine,Monique Elmaleh,Nicole Fabien,Albert Faye,Isabelle Hau,Véronique Hentgen,Thérèsa Kwon,Ulrich Meinzer,Naïm 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
摘要
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.To identify central nervous system (CNS) disease biomarkers of j-NPSLE.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.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).CSF IFN-α and neopterin constitute promising biomarkers useful in the diagnosis and monitoring of activity in j-NPSLE.