M04.3
VEXAS syndrome
M04.3 (also written M043) is a billable ICD-10-CM diagnosis code for VEXAS syndrome. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 5 codes in category M04 (Autoinflammatory syndromes). The Alphabetic Index lists it under 2 entries. Its coding notes include a Code also instruction and 11 Excludes2 entries.
Code also
Two codes may be needed to describe the condition. This note gives no sequencing order.
- any associated conditions
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Also from chapter/block level (10 notes)
- arthropathic psoriasis (L40.5-)
- certain conditions originating in the perinatal period (P04-P96)
- certain infectious and parasitic diseases (A00-B99)
- compartment syndrome (traumatic) (T79.A-)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Care settings
How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.
Home Health Primary
Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the Musculoskeletal Rehabilitation clinical group.
Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026Inclusion terms
Conditions classified to this code.
- Vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic syndrome
Notes
Also from chapter/block level (1 note)
- Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition
Alphabetic Index terms
Terms the official Alphabetic Index lists for this code. 2 entries