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What Is VEXAS Syndrome? | Everyday Health

A rare, recently discovered inflammatory disease may strike more people than previously thought, a new study suggests.

Scientists first reported the discovery of a disease they dubbed VEXAS syndrome in a paper published in 2020 in The New England Journal of Medicine (NEJM). At the time, they only identified 25 men in the United States with the condition — and no women. All had similar mutations in a gene called UBA1.

Now, a new paper published in JAMA on January 24, suggests that VEXAS, while still rare, is much more common than scientists initially believed, impacting roughly 1 in 13,600 people. The researchers also found that VEXAS is even more common in older individuals and affects both men and women: Among those 50 and older, researchers estimate a VEXAS prevalence of about 1 in 4,300 men and 1 in 26,200 women.

VEXAS most often occurs in people diagnosed with other autoimmune conditions including lupus, rheumatoid arthritis, and blood cancers.

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