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Overview of Hereditary Periodic Fever Syndromes

by Stephen E. Goldfinger, MD

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

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