M04.3: VEXAS syndrome

New code · FY2027

Billable: use this code on claims.

• New code, effective October 1, 2026 (FY2027). Before that, this condition was coded as M04.8.

Code breakdown

Coding notes

Inclusion terms

• Vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic syndrome

Code also

• any associated conditions

Excludes2 (from M04)

• Crohn's disease (K50.-)

Medicare coverage

M04.3 is listed as a covered diagnosis in 5 Medicare billing and coding articles:

• A56784 Billing and Coding: Pulmonary Stress Testing
• A57837 Billing and Coding: MolDX: Next-Generation Sequencing Lab-Developed Tests for Myeloid Malignancies and Suspected Myeloid Malignancies
• A57873 Billing and Coding: MolDX: Next-Generation Sequencing Lab-Developed Tests for Myeloid Malignancies and Suspected Myeloid Malignancies
• A57878 Billing and Coding: MolDX: Next-Generation Sequencing Lab-Developed Tests for Myeloid Malignancies and Suspected Myeloid Malignancies
• A57891 Billing and Coding: MolDX: Next-Generation Sequencing Lab-Developed Tests for Myeloid Malignancies and Suspected Myeloid Malignancies

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All FY2027 ICD-10-CM changes

ICD-10-CM codes and descriptions are from the CMS and NCHS code files. Coverage is from the CMS Medicare Coverage Database billing and coding articles.