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Overview of Hereditary Periodic Fever Syndromes

By Stephen E. Goldfinger, MD, Harvard Medical School;Massachusetts General Hospital

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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 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

* This is the Professional Version. *