학술논문

Vasculitis and vasculopathy associated with inborn errors of immunity: an overview
Document Type
article
Source
Frontiers in Pediatrics, Vol 11 (2024)
Subject
autoinflammatory diseases
DADA2
SAVI
monogenic lupus
haploinsufficiency A20
vasculopathy
Pediatrics
RJ1-570
Language
English
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.