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Interferon signature (IS) is a reliable method to measure interferon\u2010stimulated gene expression which is commonly raised in systemic lupus erythematosus (SLE) and in adult pre\u2010lupus cases. Between 2022 and 2024, IS analysis was performed on 61 children, 17 with ITP\u2010ANA+, 15 with ITP\u2010ANA\u2212, 15 with SLE and 14 with juvenile idiopathic arthritis. IS was positive in 14\/17 children (82%), with a median score of 17 (1.2\u2013143). This median IS was significantly higher than in the ITP\u2010ANA\u2212 group (2.8, 1\u201341, <jats:italic>p<\/jats:italic>\u2009=\u20090.03) and lower than in the SLE group (37.5, 2.1\u2013129, <jats:italic>p<\/jats:italic>\u2009=\u20090.04). Among ITP\u2010ANA+ children, IS elevation was associated with age &gt;10\u2009years at ITP diagnosis, newly diagnosed or persistent ITP, positive anti\u2010extractable nuclear antigen antibodies and the absence of hydroxychloroquine treatment. Hydroxychloroquine significantly reduced IS values in three children with pre\u2010 and post\u2010treatment scores available. The involvement of type I interferon signalling in childhood ITP\u2010ANA+ highlights a distinct pathogenic pathway and IS appears as a pertinent biomarker to identify patients at risk of progression to SLE. The introduction of hydroxychloroquine in these patients could help prevent the high morbidity of SLE at adult age.<\/jats:p>","DOI":"10.1111\/bjh.20241","type":"journal-article","created":{"date-parts":[[2025,7,5]],"date-time":"2025-07-05T04:55:01Z","timestamp":1751691301000},"page":"543-551","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Type I interferon as a pre\u2010lupus marker in anti\u2010nuclear antibody\u2010associated childhood immune thrombocytopenic purpura"],"prefix":"10.1111","volume":"207","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4470-1023","authenticated-orcid":false,"given":"J\u00e9r\u00f4me","family":"Granel","sequence":"first","affiliation":[{"name":"Paediatric Clinical Immunology, Pellegrin Hospital, CIC1401, INSERM CICP Bordeaux University Hospital  Bordeaux France"},{"name":"Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto\u2010Immunes de l'Enfant (CEREVANCE)  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