Journal Article

DKFZ-2025-01667

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.

Mekinian, A. M. ; Georgin-Lavaille, S. ; Ferrada, M. A. ; Savic, S. ; Koster, M. J. ; Kosmider, O. ; Comont, T. ; Heilblig, M. ; Arostegui, J. I. ; Bosco, A. ; Bourguiba, R. ; Calvo, K. R. ; Cargo, C. ; Cattaneo, C. ; Chasset, F. ; Coelho, H. ; Campochiaro, C. ; Crisafulli, F. ; Ducharme-Benard, S. ; Faria, R. ; Franceschini, F. ; Frassi, M. ; Groarke, E. M. ; Gurnari, C. ; Hakobyan, Y. ; Jamilloux, Y. ; Jurcut, C. ; Kirino, Y. ; Kulasekararaj, A. ; Kunimoto, H. ; Madigan, L. M. ; Mann, H. F. ; Marvisi, C. ; Milchert, M. ; Morais, S. ; Sockel, K.DKFZ* ; Muratore, F. ; Nakajima, H. ; Patnaik, M. M. ; Regadas, L. ; Robin, M. ; Rutgers, A. ; Salvarani, C. ; Sammel, A. M. ; Seebach, J. ; Sujobert, P. ; Tomelleri, A. ; Urbanski, G. ; Vandergheynst, F. ; Vieira, R. ; Viswanatha, D. S. ; Więsik-Szewczyk, E. ; Diral, E. ; Terrier, B. ; Patel, B. A. ; Fenaux, P. ; Grayson, P. C. ; Beck, D. B. ; International VEXAS working group ; with endorsement of EuroBloodNet, t. E. R. N. i. R. H. D. (Collaboration Author) ; Mann, H. (Contributor) ; Terrier, B. (Contributor) ; Chasset, F. (Contributor) ; Georgin Lavialle, S. (Contributor) ; Tomelleri, A. (Contributor) ; Corrado, C. (Contributor) ; Salvarani, C. (Contributor) ; Crisafulli, F. (Contributor) ; Franceschini, F. (Contributor) ; Frassi, M. (Contributor) ; Kirino, Y. (Contributor) ; Więsik-Szewczyk, E. (Contributor) ; Milchert, M. (Contributor) ; Faria, R. (Contributor) ; Jurcut, C. (Contributor) ; Seebach, J. (Contributor) ; Savic, S. (Contributor) ; Beck, D. (Contributor) ; Madigan, L. (Contributor) ; Koster, M. (Contributor) ; Mrinal, P. (Contributor) ; Kosmider, O. (Contributor) ; Sujobert, P. (Contributor) ; Arostegui, J. I. (Contributor) ; Cargo, C. (Contributor) ; Viswanatha, D. (Contributor) ; Hagopian, Y. (Contributor) ; Heilblig, M. (Contributor) ; Fenaux, P. (Contributor) ; Comont, T. (Contributor) ; Alessandro, B. (Contributor) ; Cattaneo, C. (Contributor) ; Diral, E. (Contributor) ; Morais, S. (Contributor) ; Kulasekarara, A. (Contributor) ; Groarke, E. (Contributor) ; Calvo, K. (Contributor) ; Bhavisha, P. (Contributor) ; Sammel, A. (Contributor) ; Mekinian, A. (Contributor) ; Terrier, B. (Contributor) ; Robin, M. (Contributor) ; Georgin Lavialle, S. (Contributor) ; Sockel, K. (Contributor)DKFZ* ; Jamilloux, Y. (Contributor) ; Gurnari, C. (Contributor) ; Coelho, H. (Contributor) ; Vieira, R. (Contributor) ; Bourguiba, R. (Contributor) ; Ferrada, M. (Contributor) ; Grayson, P. (Contributor)

2026
Wiley Hoboken, NJ

This record in other databases:  

Please use a persistent id in citations: doi:10.1002/art.43287

Abstract: Vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.Given the variability in disease presentation and the limited number of studies to date, no clinical documents currently exist to provide guidance to health care providers about the management of VEXAS. To address this gap, we formed an international multidisciplinary panel of VEXAS experts.Through formalized meetings and a voting process, the group developed consensus clinical guidance considerations for the management of VEXAS. These considerations offer practical advice on several key topics: (1) clinical features of VEXAS, (2) UBA1 screening methods, (3) the diagnosis of myelodysplastic syndromes (MDSs) in patients with VEXAS, and (4) prognosis and management. The aim is to provide expert guidance on which patients to test, how to test for VEXAS, how to approach MDS in the context of VEXAS, and considerations for management.This work marks the first formal international consensus guidance for VEXAS and is intended to be used as a resource for clinicians seeking to understand the disease and its management.

Note: 2026 Mar;78(3):509-522

---

Contributing Institute(s):

1. DKTK Koordinierungsstelle München (MU01)

Research Program(s):

1. 899 - ohne Topic (POF4-899) (POF4-899)

Appears in the scientific report 2025

---

Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Current Contents - Life Sciences ; DEAL Wiley ; Ebsco Academic Search ; Essential Science Indicators ; IF >= 10 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

---