M04.2 Cryopyrin-associated periodic syndromes

Billable ICD-10-CM code, FY2026.

Applicable to

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

Applies from M04 Autoinflammatory syndromes

Excludes2 (not included here)

• Crohn's disease (K50.-)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 94 — Systemic Lupus Erythematosus and Other Specified Systemic Connective Tissue Disorders
• CMS-HCC V22: HCC 40 — Rheumatoid Arthritis and Inflammatory Connective Tissue Disease
• RxHCC V08: HCC 83 — Rheumatoid Arthritis and Other Inflammatory Polyarthropathy
• ESRD V24: HCC 40

MS-DRG index

Defines the logic of DRG(s) 545-547 in MDC 08.

Approximate ICD-9-CM (GEMs)

• 710.8 Other specified diffuse diseases of connective tissue (approximate)

Code annotations containing back-references to M04.2

Back-references to M04.2

• Type 2 Excludes: L50.2 Urticaria due to cold and heat ("familial cold urticaria (M04.2)")

Back-references to M04

• Code Also: E85.0 Non-neuropathic heredofamilial amyloidosis ("autoinflammatory syndromes (M04.-)")

Diagnosis Index entries for M04.2

• 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

Nearby codes

• M04 Autoinflammatory syndromes
• M04.1 Periodic fever syndromes
• M04.2 Cryopyrin-associated periodic syndromes (this code)
• M04.8 Other autoinflammatory syndromes
• M04.9 Autoinflammatory syndrome, unspecified