Hereditary periodic fever syndromes are a subset of hereditary autoinflammatory 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 include:
Tumor necrosis factor (TNF) receptor–associated periodic syndrome
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome
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
Sporadic forms of the above hereditary periodic fever syndromes are possible.
