ICD-10-CM 2027
ICD-10 code for PFAPA
From the official ICD-10-CM alphabetic index entry “PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome)”. Page updated September 29, 2026.
About coding PFAPA
The ICD-10-CM code for PFAPA is M04.8 (Other autoinflammatory syndromes).
Within M04, choose M04.8 (Other autoinflammatory syndromes) only when documentation doesn't support a more specific option: periodic fever syndromes (M04.1), Cryopyrin-associated periodic syndromes (M04.2), VEXAS syndrome (M04.3) and autoinflammatory syndrome, unspecified (M04.9).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 545 (Connective Tissue Disorders with MCC, relative weight 2.3892), DRG 546 (Connective Tissue Disorders with CC, relative weight 1.1745) and DRG 547 (Connective Tissue Disorders without CC/MCC, relative weight 0.7526), in MDC 08 (Diseases and Disorders of the Musculoskeletal System and Connective Tissue), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about PFAPA ICD-10 codes
What is the ICD-10 code for PFAPA?
- M04.8 — Other autoinflammatory syndromes.
Is M04.8 billable?
- Yes. M04.8 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can M04.8 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict M04.8 as a principal diagnosis.
Is M04.8 a CC or MCC?
- No. M04.8 is neither a CC nor an MCC under MS-DRG v44.0.