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Article

VEXAS syndrome in adults: A narrative review of genomic pathogenesis, multisystem phenotypes, diagnostic algorithms, and emerging therapeutic strategies.

Autoimmunity reviews - 1 May 2026

Vitale Antonio, Cifuentes-González Carlos, Gentile Martina, Cataldi Giulia, Agrawal Kajal, Agrawal Rupesh, Conforti Alessandro, Cantarini Luca

Abstract excerpt

AIM: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently defined adult-onset autoinflammatory disorder caused by somatic mutations in the UBA1 gene. This narrative review synthesizes current insights into its epidemiology, pathogenesis, clinical spectrum, diagnostic strategies, and emerging therapeutic approaches, emphasizing data from the French and AIDA registries. METHOD: We...

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Source

Identifiers

PMID: 41871819
DOI: 10.1016/j.autrev.2026.104030

Genes

UBA1
AIDA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

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