American College of Rheumatology Issues New Guidance for Diagnosis and Management of VEXAS Syndrome

Expert Statement Developed by International VEXAS Working Group

ATLANTA – The American College of Rheumatology (ACR) has released a guidance statement for the diagnosis and management of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. This pioneering document was developed by the International VEXAS Working Group, a global panel of multidisciplinary experts in rheumatology, hematology, immunology, and genetics.

VEXAS syndrome is a recently identified, life-threatening autoinflammatory condition caused by mutations in the UBA1 gene. It presents a complex array of symptoms, including systemic inflammation, blood-related complications, and multi-organ involvement, often leading to delayed or missed diagnoses.

The new guidance provides clinicians with evidence-informed recommendations and expert consensus on:

• Diagnostic criteria for identifying VEXAS syndrome in patients with unexplained systemic inflammation and hematologic abnormalities
• Recommended laboratory and genetic testing protocols, including the role of UBA1 mutation screening
• Management strategies, including immunosuppressive therapies, hematologic interventions, and emerging treatment options
• Multidisciplinary care coordination to address the syndrome’s complex clinical manifestations

“This guidance is a critical step forward in helping clinicians recognize and manage VEXAS syndrome earlier and more effectively,” said Arsene M. Mekinian, MD, member of the International VEXAS Working Group. “Given the severity and complexity of this condition, timely diagnosis and coordinated care are essential to improving patient outcomes.”

The guidance statement is now available on the ACR website and was recently published in an issue of Arthritis & Rheumatology.

Media Contact
Teri Arnold
tarnold@rheumatology.org
757-272-7002 (cell)

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About the American College of Rheumatology
The American College of Rheumatology (ACR) is an international medical society representing nearly 10,000 rheumatologists and rheumatology professionals with a mission to empower rheumatology professionals to excel in their specialty. In doing so, the ACR offers education, research, advocacy and practice management support to help its members continue their innovative work and provide quality patient care. Rheumatologists are experts in the diagnosis, management and treatment of more than 100 different types of arthritis and rheumatic diseases.