Nous sommes heureux d’annoncer la publication du travail de Jérôme Granel et Helder Fernandes intitulé « Type I interferon as a pre-lupus marker in anti-nuclearantibody-associated childhood immune thrombocytopenic purpura » dans la revue British Journal of Haematology.
Abstract:
Paediatric-onset anti-nuclear antibody-associated immune thrombocytopenic pur-pura (ITP-ANA+) is a pre-lupus condition. Interferon signature (IS) is a reliablemethod to measure interferon-stimulated gene expression which is commonly raisedin systemic lupus erythematosus (SLE) and in adult pre-lupus cases. Between 2022and 2024, IS analysis was performed on 61 children, 17 with ITP-ANA+, 15 with ITP-ANA−, 15 with SLE and 14 with juvenile idiopathic arthritis. IS was positive in 14/17children (82%), with a median score of 17 (1.2–143). This median IS was significantlyhigher than in the ITP-ANA− group (2.8, 1–41, p = 0.03) and lower than in the SLEgroup (37.5, 2.1–129, p = 0.04). Among ITP-ANA+ children, IS elevation was associ-ated with age >10 years at ITP diagnosis, newly diagnosed or persistent ITP, positiveanti-extractable nuclear antigen antibodies and the absence of hydroxychloroquinetreatment. Hydroxychloroquine significantly reduced IS values in three childrenwith pre- and post-treatment scores available. The involvement of type I interferonsignalling in childhood ITP-ANA+ highlights a distinct pathogenic pathway and ISappears as a pertinent biomarker to identify patients at risk of progression to SLE.The introduction of hydroxychloroquine in these patients could help prevent thehigh morbidity of SLE at adult age.
Lien: https://onlinelibrary.wiley.com/doi/epdf/10.1111/bjh.20241