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=Volume 11 - 2023

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 the innate immunity, characterized by apparently unprovoked recurrent flares of systemic inflammation usually characterized by fever associated to clinical manifestation involving mainly the musculoskeletal, mucocutaneous, gastrointestinal and nervous systems. Several conditions present a various, sometimes prominent, involvement of the vascular system too with features of vasculitis characterized by variable target vessel involvement and organ damage. Here we report a systematic review of vasculitis and vasculopathies associated with inborn errors of immunity.