Most patients develop symptoms during childhood; < 10% develop symptoms after age 18. Disorders best characterized are
Other disorders include
-
The hereditary cryopyrin-associated periodic syndromes (cryopyrinopathies): Familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease (NOMID)
-
PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome
-
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome, which may not be hereditary