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 across all age groups is
A similar disorder that causes episodes of fever but is not thought to be hereditary is
PFAPA syndrome PFAPA Syndrome PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome causes recurring episodes of fever that last 3 to 6 days, mouth sores (stomatitis), a sore throat (pharyngitis)... read more (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, rilonacept, 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. nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and anakinra may help relieve symptoms during attacks. Anakinra or canakinumab is given to prevent 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 Pyoderma Gangrenosum Pyoderma gangrenosum is a chronic inflammatory skin disease of unknown cause that results in large sores forming on the skin. (See also Introduction to Hypersensitivity Reactions and Inflammatory... read more , and acne Acne Acne is a common skin condition causing pimples and other abnormalities on the face and upper torso. Acne is caused by a buildup of dead skin cells, bacteria, and dried sebum that blocks the... read more ): This syndrome causes inflamed joints, skin ulcers, and acne. By puberty, the arthritis tends to subside and the skin problems are the main symptoms. Anakinra or etanercept may help. Acne Treatment Acne is a common skin condition causing pimples and other abnormalities on the face and upper torso. Acne is caused by a buildup of dead skin cells, bacteria, and dried sebum that blocks the... read more 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.