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Article

Hypomethylating agents in vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS): A systematic review.

British journal of haematology - 1 Mar 2026

Hoff Fieke W, Trikha Roochi, Groarke Emma M, Patel Bhavisha A

Abstract excerpt

VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is an X-linked, systemic, haemato-inflammatory syndrome caused by somatic mutations in the UBA1 gene. No standardized treatment guidelines exist, but evidence is emerging that treatment with hypomethylating agents (HMAs) can induce improvement of the inflammatory symptoms, reverse cytopenia, enable weaning of the corticosteroids and, in...

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Identifiers

PMID: 41531276
DOI: 10.1111/bjh.70302
PMCID: PMC12995528

Genes

UBA1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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