Case Report: VEXAS Syndrome: From Mild Symptoms to Life-Threatening Macrophage Activation Syndrome

Recently, a novel disorder coined VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was identified in patients with adult-onset inflammatory syndromes, often accompanied by myelodysplastic syndrome1. All patients had myeloid lineage-restricted somatic mutations in UBA1 affect...

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Bibliographic Details

Published in:	Frontiers in Immunology
Main Authors:	Frederik Staels, Albrecht Betrains, F. J. Sherida H. Woei-A-Jin, Nancy Boeckx, Marielle Beckers, An Bervoets, Mathijs Willemsen, Barbara Neerinckx, Stephanie Humblet-Baron, Daniel Engelbert Blockmans, Steven Vanderschueren, Rik Schrijvers
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2021-04-01
Subjects:	primary immunodeficiencies autoinflammation treatment reactions myelodysplasia ubiquitination
Online Access:	https://www.frontiersin.org/articles/10.3389/fimmu.2021.678927/full