AUTHOR=Federici Silvia , Cinicola Bianca Laura , La Torre Francesco , Castagnoli Riccardo , Lougaris Vassilios , Giardino Giuliana , Volpi Stefano , Caorsi Roberta , Leonardi Lucia , Corrente Stefania , Soresina Annarosa , Cancrini Caterina , Insalaco Antonella , Gattorno Marco , De Benedetti Fabrizio , Marseglia Gian Luigi , Del Giudice Michele Miraglia , Cardinale Fabio TITLE=Vasculitis and vasculopathy associated with inborn errors of immunity: an overview JOURNAL=Frontiers in Pediatrics VOLUME=11 YEAR=2024 URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2023.1258301 DOI=10.3389/fped.2023.1258301 ISSN=2296-2360 ABSTRACT=

Systemic autoinflammatory diseases (SAIDs) are disorders of innate immunity, which are characterized by unprovoked recurrent flares of systemic inflammation often characterized by fever associated with clinical manifestations mainly involving the musculoskeletal, mucocutaneous, gastrointestinal, and nervous systems. Several conditions also present with varied, sometimes prominent, involvement of the vascular system, with features of vasculitis characterized by variable target vessel involvement and organ damage. Here, we report a systematic review of vasculitis and vasculopathy associated with inborn errors of immunity.