Skip to content
Dx

ICD-10-CM 2027 diagnosis code

M04.2Cryopyrin-associated periodic syndromes

✓ Billable / specific

M04.2 is a valid, billable ICD-10-CM code for cryopyrin-associated periodic syndromes. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Looking up by condition? See ICD-10 code for CINCA, ICD-10 code for NOMID.

Code last changed in FY2017 (effective October 1, 2016). Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About M04.2

M04.2 is the ICD-10-CM diagnosis code for Cryopyrin-associated periodic syndromes. It belongs to category M04 (autoinflammatory syndromes), block M04 (autoinflammatory syndromes) and chapter 13 (diseases of the musculoskeletal system and connective tissue). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within M04 (autoinflammatory syndromes), M04.2 is specifically for Cryopyrin-associated periodic syndromes. Related codes cover periodic fever syndromes (M04.1), VEXAS syndrome (M04.3), other autoinflammatory syndromes (M04.8) and autoinflammatory syndrome, unspecified (M04.9).

It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.

In MS-DRG v44.0, it is part of the grouping logic for DRG 545 (Connective Tissue Disorders with MCC, relative weight 2.3892), DRG 546 (Connective Tissue Disorders with CC, relative weight 1.1745) and DRG 547 (Connective Tissue Disorders without CC/MCC, relative weight 0.7526), in MDC 08 (Diseases and Disorders of the Musculoskeletal System and Connective Tissue), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It was added in FY2017, effective October 1, 2016.

Before ICD-10, this condition was coded in ICD-9-CM as 710.8 (other specified diffuse diseases of connective tissue).

Coding notes

Applicable toConditions and synonyms classified to this code
  • Chronic infantile neurological, cutaneous and articular syndrome [CINCA]
  • Familial cold autoinflammatory syndrome
  • Familial cold urticaria
  • Muckle-Wells syndrome
  • Neonatal onset multisystemic inflammatory disorder [NOMID]

Notes that apply from higher levels

Instructions written at a parent level also apply to M04.2.

› From M04 Autoinflammatory syndromes
Excludes2
  • Crohn's disease (K50.-)

Broader instructions also apply from Chapter 13: Diseases of the musculoskeletal system and connective tissue.

Alphabetic index entries

8 entries

Terms in the official ICD-10-CM index that lead to M04.2.

Codes whose notes reference M04.2

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

ICD-9-CM equivalent

Converter →
  • 710.8Other specified diffuse diseases of connective tissueapproximate

From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2017 (effective 10/1/2016): Added
  • No changes since FY2017.

Common questions about M04.2

Is M04.2 billable?

Yes. M04.2 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can M04.2 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict M04.2 as a principal diagnosis.

Is M04.2 a CC or MCC?

No. M04.2 is neither a CC nor an MCC under MS-DRG v44.0.

What DRG does M04.2 group to?

M04.2 is used in the MS-DRG v44.0 logic for MS-DRG 545 (Connective Tissue Disorders with MCC), MS-DRG 546 (Connective Tissue Disorders with CC) and MS-DRG 547 (Connective Tissue Disorders without CC/MCC). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.

What is the ICD-9 code for M04.2?

The CMS General Equivalence Mappings map M04.2 to ICD-9-CM 710.8.