2026 ICD-10-CM Diagnosis Code M04.2Cryopyrin-associated periodic syndromes
ICD-10-CM Codes›M00–M99›M04›M04
- Billable — Valid for Submission
- Risk Adjusts — HCC 94
- Chronic Condition
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.
For Medicare Advantage risk adjustment, M04.2 maps to CMS-HCC Category 94 (Systemic Lupus Erythematosus and Other Specified Systemic Connective Tissue Disorders) under the V28 model, adding a risk factor of about 0.268 for a community, non-dual, aged beneficiary in payment year 2026.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
M04.2 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2026.
Source: CMS Payment Year 2026 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
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]
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Disorder (of) See Also: Disease;
Syndrome See Also: Disease;
familial cold M04.2
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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.
Code History & ChangesHistory
Replacement M04.2 replaces the following previously assigned code(s):
- L50.2 - Urticaria due to cold and heat
Questions About M04.2Overview
What is the ICD-10 code for cryopyrin-associated periodic syndromes?
The ICD-10-CM code for cryopyrin-associated periodic syndromes is M04.2 (sometimes written as M042). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.
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.
What HCC is M04.2?
M04.2 (cryopyrin-associated periodic syndromes) maps to CMS-HCC Category 94 (Systemic Lupus Erythematosus and Other Specified Systemic Connective Tissue Disorders), commonly written as HCC 94, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2026. It mapped to HCC 40 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 83.
Does M04.2 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, M04.2 adds a risk adjustment factor of about 0.268 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.196 to 0.297 depending on the payment segment). A more severe related category (HCC 93) supersedes it when both are reported. See the full factor table on the HCC 94 category page.
