ICD-10-CM 2027
ICD-10 code for Hyperimmunoglobulin D syndrome
From the official ICD-10-CM alphabetic index entry “Syndrome › hyperimmunoglobulin D”. Page updated September 29, 2026.
About coding Hyperimmunoglobulin D syndrome
The ICD-10-CM code for Hyperimmunoglobulin D syndrome is M04.1 (Periodic fever syndromes).
Within M04 (autoinflammatory syndromes), M04.1 is specifically for periodic fever syndromes. Related codes cover Cryopyrin-associated periodic syndromes (M04.2), VEXAS syndrome (M04.3), other autoinflammatory syndromes (M04.8) 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 Hyperimmunoglobulin D syndrome ICD-10 codes
What is the ICD-10 code for Hyperimmunoglobulin D syndrome?
- M04.1 — Periodic fever syndromes.
Is M04.1 billable?
- Yes. M04.1 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can M04.1 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict M04.1 as a principal diagnosis.
Is M04.1 a CC or MCC?
- No. M04.1 is neither a CC nor an MCC under MS-DRG v44.0.