DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

M04 Autoinflammatory syndromes

  • Header · not billable

M04 is not billable. The category heads 5 billable codes in FY2027: the 5 codes directly below it.

FY2027 changes under M04: 1 billable code added. All FY2027 changes.

Index → code

  • DIRA (deficiency of interleukin 1 receptor antagonist) → M04.8
  • NOMID (neonatal onset multisystemic inflammatory disorder) → M04.2
  • PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome) → M04.8

Where it sits

  1. Chapter 13 Diseases of the musculoskeletal system and connective tissue (M00-M99)
  2. M04 Autoinflammatory syndromes (M04)
  3. M04 Autoinflammatory syndromes
Billable codes
5
Subdivisions
5
CC / MCC codes
0 / 0
HCC V28 codes
4
New FY2027
1
Index entries
33

Codes in M04

  1. M04.1
    Periodic fever syndromes
    Billable94
    Inclusion terms 4

    Inclusion terms

    • Familial Mediterranean fever
    • Hyperimmunoglobin D syndrome
    • Mevalonate kinase deficiency
    • Tumor necrosis factor receptor associated periodic syndrome [TRAPS]
  2. M04.2
    Cryopyrin-associated periodic syndromes
    Billable94
    Inclusion terms 5

    Inclusion terms

    • Chronic infantile neurological, cutaneous and articular syndrome [CINCA]
    • Familial cold autoinflammatory syndrome
    • Familial cold urticaria
    • Muckle-Wells syndrome
    • Neonatal onset multisystemic inflammatory disorder [NOMID]
  3. M04.3
    VEXAS syndrome New FY2027
    Billable
    Inclusion terms 1, Code also 1

    Inclusion terms

    • Vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic syndrome

    Code also

    • any associated conditions
  4. M04.8
    Other autoinflammatory syndromes
    Billable94
    Inclusion terms 5

    Inclusion terms

    • 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]
  5. M04.9
    Autoinflammatory syndrome, unspecified
    Billable94

Sources of each column.

Instructions

At M04

Excludes2

  • Crohn's disease (K50.-) K50

Notes that also apply

Instructions for the group M00-M25 (Includes 1): printed on the chapter 13 page.

Chapter 13 instructions (Notes 1, Excludes2 10): printed on the chapter 13 page.

Index entries

  • TRAPS (tumor necrosis factor receptor associated periodic syndrome) → M04.1
  • CINCA (chronic infantile neurological, cutaneous and articular syndrome) → M04.2
  • NOMID (neonatal onset multisystemic inflammatory disorder) → M04.2
  • DIRA (deficiency of interleukin 1 receptor antagonist) → M04.8
  • PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne syndrome) → M04.8
  • PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome) → M04.8
  • Deficiency, deficientmevalonate kinase → M04.1
  • Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor)periodic (Mediterranean) → M04.1
  • Syndromehyperimmunoglobulin D → M04.1
  • Syndromeperiodic fever → M04.1
  • Syndrometumor necrosis factor receptor associated periodic (TRAPS) → M04.1
  • Disorder (of)neonatal onset multisystemic inflammatory (NOMID) → M04.2
  • Syndromecryopyrin-associated periodic → M04.2
  • Syndromefamilial cold autoinflammatory → M04.2
  • SyndromeMuckle-Wells → M04.2
  • Urticariafamilial cold → M04.2
  • Syndromevacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic (VEXAS) → M04.3
  • SyndromeVEXAS (vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic) → M04.3
  • Deficiency, deficientof interleukin 1 receptor antagonist [DIRA] → M04.8
  • SyndromeBlau → M04.8
  • SyndromeMajeed → M04.8
  • Syndromeperiodic fever, aphthous stomatitis, pharyngitis, and adenopathy [PFAPA] → M04.8
  • Syndromepyogenic arthritis, pyoderma gangrenosum, and acne [PAPA] → M04.8
  • SyndromeYao → M04.8
  • Disease, diseasedautoinflammatory → M04.9
  • Syndromeautoinflammatory → M04.9
  • Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute)in (due to)Mediterranean fever, familial → M04.1
  • Fever (inanition) (of unknown origin) (persistent) (with chills) (with rigor)Mediterraneanfamilial → M04.1
  • Mediterraneanfeverfamilial → M04.1
  • Syndromechronicinfantile neurological, cutaneous and articular (CINCA) → M04.2
  • Disease, diseasedautoinflammatoryNOD2-associated → M04.8
  • Disease, diseasedautoinflammatoryspecified type NEC → M04.8
  • Syndromeautoinflammatoryspecified type NEC → M04.8

NCHS Alphabetic Index. Index first, then the Tabular List.