Overview of Hereditary Periodic Fever Syndromes
Hereditary periodic fever syndromes are hereditary disorders characterized by recurrent fever and other symptoms that are not explained by other causes.
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