1. M04 Autoinflammatory syndromes

    Excludes2

    • Crohn's disease (K50.-)
    1. M04.1 Periodic fever syndromes

      Inclusion term(s):

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

      Inclusion term(s):

      • 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. M04.8 Other autoinflammatory syndromes

      Inclusion term(s):

      • 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]
    4. M04.9 Autoinflammatory syndrome, unspecified

Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.