Autoinflammatory syndromes (M04)

M04Autoinflammatory syndromes
Excludes 2: Crohn's disease (K50.-)

M04.1Periodic fever syndromes
› Familial Mediterranean fever
› Hyperimmunoglobin D syndrome
› Mevalonate kinase deficiency
› Tumor necrosis factor receptor associated periodic syndrome [TRAPS]

M04.2Cryopyrin-associated periodic syndromes
› Chronic infantile neurological, cutaneous and articular syndrome [CINCA]
› Familial cold autoinflammatory syndrome
› Familial cold urticaria
› Muckle-Wells syndrome
› Neonatal onset multisystemic inflammatory disorder [NOMID]

M04.8Other autoinflammatory syndromes
› 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]

M04.9Autoinflammatory syndrome, unspecified