2026 ICD-10-CM Diagnosis Code M04.2Cryopyrin-associated periodic syndromes

ICD-10-CM Codes›M00–M99›M04›M04

ICD-10-CM M04.2
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

M04.2 is a billable ICD-10-CM diagnosis code for cryopyrin-associated periodic syndromes. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 545 through 547. Coders also document this condition as chronic infantile neurological, cutaneous and articular syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Autoinflammatory syndromes.

Code Identity

ICD-10-CM Code
M04.2
Billable Status
Yes — Valid for Submission
Code Describes
Cryopyrin-associated periodic syndromes
Short Description
Cryopyrin-associated periodic syndromes
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 CodeM04.2Cryopyrin-associated periodic syndromes

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Chronic infantile neurological, cutaneous and articular syndrome
  • Cryopyrin associated periodic syndrome
  • F12-associated cold autoinflammatory syndrome
  • Familial cold urticaria
  • Urticaria caused by cold

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Chronic infantile neurological, cutaneous and articular syndrome CINCA
  • Familial cold autoinflammatory syndrome
  • Familial cold urticaria
  • Muckle-Wells syndrome
  • Neonatal onset multisystemic inflammatory disorder NOMID

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • CINCA(chronic infantile neurological, cutaneous and articular syndrome)
    • Disorder(of)
      • neonatal onset multisystemic inflammatory (NOMID)
    • NOMID(neonatal onset multisystemic inflammatory disorder)
    • Syndrome
      • chronic
        • infantile neurological, cutaneous and articular (CINCA)
    • Syndrome
      • cryopyrin-associated periodic
    • Syndrome
      • familial cold autoinflammatory
    • Syndrome
      • Muckle-Wells
    • Urticaria
      • familial cold

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MUS036
Autoinflammatory syndromes
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Cryopyrin-associated periodic syndromes

Cryopyrin-associated periodic syndromes (CAPS) are a group of conditions that have overlapping signs and symptoms and the same genetic cause. The group includes three conditions known as familial cold autoinflammatory syndrome type 1 (FCAS1), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disorder (NOMID).

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert M04.2 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
710.8 Diff connect tis dis NEC
Approximate The match is approximate rather than exact.

Code History & ChangesHistory

Replacement M04.2 replaces the following previously assigned code(s):

  • L50.2 - Urticaria due to cold and heat
FY 2018AddedAdded to the ICD-10-CM code setEffective October 1, 2017.
FY 2019–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About M04.2Overview

Is M04.2 (Autoinflammatory syndromes) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report cryopyrin-associated periodic syndromes on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does M04.2 group to?

When cryopyrin-associated periodic syndromes is the principal diagnosis on an inpatient stay, it groups to MS-DRG 545, 546, 547, with relative weights from 0.8362 to 2.4817 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of M04.2?

Under the General Equivalence Mappings, cryopyrin-associated periodic syndromes converts to ICD-9-CM 710.8 (diff connect tis dis NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.