Overview of Hereditary Periodic Fever Syndromes

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

• Familial Mediterranean fever

• Hyper-IgD syndrome

• 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