Hereditary periodic fever syndromes are hereditary disorders that periodically cause episodes of fever and other symptoms that are not due to usual childhood infections or any other obvious disorder. The more common of these syndromes include

• Familial Mediterranean fever
• PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome

Other less common syndromes include the following:

• Hereditary cryopyrinopathies: These include familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease (NOMID). Episodes of fever, a rash, and joint pain are periodically triggered by cold temperatures.
• Hyper-IgD syndrome: This syndrome causes abdominal pain, vomiting or diarrhea, headache, joint pain, a rash, swollen lymph glands, and mouth and genital sores in addition to chills and fever.
• Tumor necrosis factor (TNF) receptor–associated periodic syndrome: This syndrome causes periodic attacks of muscle pain and swelling in the arms and legs, abdominal pain, joint pain, and rash in addition to fever.
• PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne): This syndrome causes inflamed joints, skin ulcers, and acne.

Symptoms usually begin during childhood. Fewer than 10% of people develop symptoms after age 18. People periodically have attacks of fever and inflammation but feel well between attacks.

Last full review/revision January 2009 by Stephen E. Goldfinger, MD