AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2026 Quarter 4; New/Revised ICD-10-CM Codes

VEXAS Syndrome

Code M04.3 , VEXAS syndrome, has been created at category M04 , Autoinflammatory syndromes, to specifically identify Vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. VEXAS syndrome is an autoinflammatory condition in which the immune system attacks the body by mistake and causes inflammation to spread all over the body. It is a rare autoinflammatory disease caused by an acquired gene mutation (UBA1) that occurs after birth primarily affecting biological males over the age of 50. Since VEXAS syndrome is acquired rather than inherited, it cannot be passed down through generations. Symptoms may include irritation and redness of the...

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