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Overview of Hereditary Periodic Fever Syndromes

By Apostolos Kontzias, MD, Assistant Professor of Medicine and Director, Autoinflammatory Clinic, Cleveland Clinic Foundation

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 most common hereditary periodic fever syndrome is

A similar disorder that causes episodes of fever but is not thought to be hereditary is

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

Other less common syndromes include the following:

  • Hereditary cryopyrin-associated periodic syndromes (cryopyrinopathies): These syndromes include familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem autoinflammatory disease (NOMID). Episodes of fever, a rash, and joint pain are periodically triggered by cold temperatures. The drugs anakinra or canakinumab, which modify how the immune system functions, may help.

  • Hyper-IgD syndrome: This syndrome causes fever, abdominal pain, headache, joint pain, a rash, and swollen lymph nodes. NSAIDs, corticosteroids, and anakinra may help relieve symptoms during attacks.

  • Tumor necrosis factor receptor–associated periodic syndrome (TRAPS): 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. Prednisone, a corticosteroid, is used to suppress symptoms during attacks. Anakinra, canakinumab, or sometimes etanercept are used for attacks.

  • PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne): This syndrome causes inflamed joints, skin ulcers, and acne. Anakinra or etanercept may help. Acne is treated with the antibiotic tetracycline or with isotretinoin.

Symptoms of hereditary periodic fever syndromes 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.

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