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If not fatal<jats:italic>in utero<\/jats:italic>, complete CHB typically requires lifelong cardiac pacing. No treatment has so far been unequivocally demonstrated to prevent or treat autoimmune CHB, and the relatively low incidence (1%\u20105%) and recurrence (12%\u201016%) rates of second\/third\u2010degree AV block add to the complexity of managing pregnancies in women with anti\u2010Ro\/La antibodies. Altogether, a better understanding of events leading to development of autoimmune CHB is needed to improve surveillance and treatment strategies. In the past decade, studies have started to look beyond the role of maternal autoantibodies in disease pathogenesis to assess other contributing factors such as foetal genetics and, more recently, immune responses in foetuses and neonates of anti\u2010Ro\/La antibody\u2010positive women. In this review, we provide an update on the epidemiology, clinical presentation and current treatment approaches of autoimmune CHB, summarize the previously proposed pathogenic mechanisms implicating maternal autoantibodies, and discuss the recent findings of type I interferon (IFN) and innate immune activation in foetuses with autoimmune CHB and in neonates of anti\u2010Ro\/La antibody\u2010positive mothers, and how these may contribute to autoimmune CHB pathogenesis.<\/jats:p>","DOI":"10.1111\/sji.12995","type":"journal-article","created":{"date-parts":[[2020,11,14]],"date-time":"2020-11-14T15:35:21Z","timestamp":1605368121000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Interferons and innate immune activation in autoimmune congenital heart block"],"prefix":"10.1111","volume":"93","author":[{"given":"Aurelie","family":"Ambrosi","sequence":"first","affiliation":[{"name":"Division of Rheumatology Department 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