M04.1 Periodic fever syndromes

Billable ICD-10-CM code, FY2026.

Applicable to

• Familial Mediterranean fever
• Hyperimmunoglobin D syndrome
• Mevalonate kinase deficiency
• Tumor necrosis factor receptor associated periodic syndrome [TRAPS]

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)

• 277.31 Familial Mediterranean fever (approximate)

Code annotations containing back-references to M04.1

Back-references to M04

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

Diagnosis Index entries for M04.1

• Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute) › in (due to) › Mediterranean fever, familial
• Deficiency, deficient › mevalonate kinase
• Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor) › Mediterranean › familial
• Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor) › periodic (Mediterranean)
• Mediterranean › fever › familial
• Syndrome › hyperimmunoglobulin D
• Syndrome › periodic fever
• Syndrome › tumor necrosis factor receptor associated periodic (TRAPS)
• TRAPS (tumor necrosis factor receptor associated periodic syndrome)

Nearby codes

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