M04.8 Other autoinflammatory syndromes

Billable ICD-10-CM code, FY2026.

Applicable to

• Blau syndrome
• Deficiency of interleukin 1 receptor antagonist [DIRA]
• Majeed syndrome
• Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome [PFAPA]
• Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome [PAPA]

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 M04.8

Back-references to M04

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

Diagnosis Index entries for M04.8

• Deficiency, deficient › of interleukin 1 receptor antagonist [DIRA]
• DIRA (deficiency of interleukin 1 receptor antagonist)
• Disease, diseased › autoinflammatory › NOD2-associated
• Disease, diseased › autoinflammatory › specified type NEC
• PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome)
• PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome)
• Syndrome › autoinflammatory › specified type NEC
• Syndrome › Blau
• Syndrome › Majeed
• Syndrome › periodic fever, aphthous stomatitis, pharyngitis, and adenopathy [PFAPA]
• Syndrome › pyogenic arthritis, pyoderma gangrenosum, and acne [PAPA]
• Syndrome › Yao

Nearby codes

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