2027 ICD-10-CM Diagnosis Code M04.3VEXAS syndrome

ICD-10-CM Codes›M00–M99›M04›M04

ICD-10-CM M04.3
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

M04.3 is a billable ICD-10-CM diagnosis code for VEXAS syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 545 through 547.

Code Identity

ICD-10-CM Code
M04.3
Billable Status
Yes — Valid for Submission
Code Describes
VEXAS syndrome
Short Description
VEXAS syndrome
Same as the full description in the CMS dataset.
Parent Code
M04
Autoinflammatory syndromes

Code Classification

ChapterM00–M99Diseases of the musculoskeletal system and connective tissue
SectionM04Autoinflammatory syndromes
CategoryM04Autoinflammatory syndromes
This CodeM04.3VEXAS syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to M04.3: its own notes plus those printed at M04, block M00-M25, and Chapter 13. A note printed at a category, block or chapter applies to every code under it.

Includes

Further defines, or gives examples of, the content of the category.

From Block M00-M25 applies to 2,249 codes
  • Disorders affecting predominantly peripheral (limb) joints

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

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

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From M04 Autoinflammatory syndromes applies to 5 codes
From Chapter 13 (M00-M99) Diseases of the musculoskeletal system and connective tissue applies to 6,687 codes
  • 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)

Code Also

A second code may be needed to fully describe the condition; the note gives no sequencing direction.

  • any associated conditions

Notes

General instructions on how these codes are used.

From Chapter 13 (M00-M99) Diseases of the musculoskeletal system and connective tissue applies to 6,687 codes
  • Use an external cause code following the code for the musculoskeletal condition, if applicable, to identify the cause of the musculoskeletal condition

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name M04.3, its category, or a range that includes it.

Code Also 1

These codes say M04.3 may also be needed to fully describe the condition. The note gives no sequencing direction.

  • E85.0 Non-neuropathic heredofamilial amyloidosis
    autoinflammatory syndromes (M04.-) names M04.-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical InformationClinical

  • VEXAS Syndrome

    an x-linked inherited condition caused by somatic mutation(s) in the uba1 gene, encoding ubiquitin-like modifier-activating enzyme 1. it is characterized by the adult onset of rheumatologic symptoms in males, including recurrent fevers, pulmonary and dermatologic inflammatory manifestations, vasculitis, arthralgias, facial chondritis and hematologic abnormalities.

Code History & ChangesHistory

New Code M04.3 was added to the ICD-10-CM code set for FY 2027, effective October 1, 2026.

Replacement M04.3 replaces the following previously assigned code(s):

  • M04.8 - Other autoinflammatory syndromes
FY 2027AddedAdded to the ICD-10-CM code setEffective October 1, 2026.
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About M04.3Overview

What is the ICD-10 code for VEXAS syndrome?

The ICD-10-CM code for VEXAS syndrome is M04.3 (sometimes written as M043). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is M04.3 (Autoinflammatory syndromes) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report VEXAS syndrome on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does M04.3 group to?

When VEXAS syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 546, 545, 547, with relative weights from 0.7160 to 2.3903 depending on complications. Higher weights mean higher Medicare reimbursement.