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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 (which may not be hereditary)
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
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