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

• Familial Mediterranean fever
• Hyper-IgD syndrome
• Tumor necrosis factor (TNF) receptor–associated periodic syndrome

Others include

• The hereditary 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

Last full review/revision February 2013 by Stephen E. Goldfinger, MD