Frontiers in Pediatrics (Jan 2024)

Vasculitis and vasculopathy associated with inborn errors of immunity: an overview

  • Silvia Federici,
  • Bianca Laura Cinicola,
  • Bianca Laura Cinicola,
  • Francesco La Torre,
  • Riccardo Castagnoli,
  • Riccardo Castagnoli,
  • Vassilios Lougaris,
  • Giuliana Giardino,
  • Stefano Volpi,
  • Roberta Caorsi,
  • Lucia Leonardi,
  • Stefania Corrente,
  • Annarosa Soresina,
  • Caterina Cancrini,
  • Caterina Cancrini,
  • Antonella Insalaco,
  • Marco Gattorno,
  • Fabrizio De Benedetti,
  • Gian Luigi Marseglia,
  • Gian Luigi Marseglia,
  • Michele Miraglia Del Giudice,
  • Fabio Cardinale

DOI
https://doi.org/10.3389/fped.2023.1258301
Journal volume & issue
Vol. 11

Abstract

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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.

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