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Review
. 2024 Jan 31:11:1258301.
doi: 10.3389/fped.2023.1258301. eCollection 2023.

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

Silvia Federici #  1 , Bianca Laura Cinicola #  2   3 , Francesco La Torre #  4 , Riccardo Castagnoli  5   6 , Vassilios Lougaris  7 , Giuliana Giardino  8 , Stefano Volpi  9 , Roberta Caorsi  9 , Lucia Leonardi  2 , Stefania Corrente  10 , Annarosa Soresina  11 , Caterina Cancrini  12   13 , Antonella Insalaco  1 , Marco Gattorno  2 , Fabrizio De Benedetti  1 , Gian Luigi Marseglia  5   6 , Michele Miraglia Del Giudice  14 , Fabio Cardinale  4
Affiliations
Review

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

Silvia Federici et al. Front Pediatr. .

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.

Keywords: DADA2; SAVI; autoinflammatory diseases; haploinsufficiency A20; monogenic lupus; vasculopathy.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Colliquated poromalacic area in the deep left temporal region, at the site of the previous hemorrhage, surrounded by a thick hemosiderin rim.
See this image and copyright information in PMC

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