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 without a clearly defined pattern of inheritance 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 medications anakinra, rilonacept, or canakinumab, which modify how the immune system functions, may help.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 medications anakinra, rilonacept, or canakinumab, which modify how the immune system functions, may help.
Hyper-IgD syndrome: This syndrome causes fever, chills, abdominal pain, headache, joint pain, a rash, and swollen lymph nodes. Nonsteroidal anti-inflammatory drugs (NSAIDs), steroids (also called glucocorticoids or corticosteroids), and anakinra may help relieve symptoms during attacks. Canakinumab or Hyper-IgD syndrome: This syndrome causes fever, chills, abdominal pain, headache, joint pain, a rash, and swollen lymph nodes. Nonsteroidal anti-inflammatory drugs (NSAIDs), steroids (also called glucocorticoids or corticosteroids), and anakinra may help relieve symptoms during attacks. Canakinumab oranakinra 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. NSAIDs are used for mild attacks. However, people usually require prednisone, a steroid, for typical attacks (fevers, joint swelling, and muscle aches that seem to affect different parts of the body at different times). Anakinra and canakinumab may be 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. NSAIDs are used for mild attacks. However, people usually require prednisone, a steroid, for typical attacks (fevers, joint swelling, and muscle aches that seem to affect different parts of the body at different times). Anakinra and canakinumab may be given to prevent attacks.
PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, and acne): This syndrome causes fever, inflamed joints, skin sores (ulcers), and acne. By puberty, the joint pain tends to subside and the skin problems are the main symptoms. Anakinra, etanercept, adalimumab, or infliximab may help. , and acne): This syndrome causes fever, inflamed joints, skin sores (ulcers), and acne. By puberty, the joint pain tends to subside and the skin problems are the main symptoms. Anakinra, etanercept, adalimumab, or infliximab may help.Acne is treated with the antibiotic tetracycline or with isotretinoin.is treated with the antibiotic tetracycline or with isotretinoin.
© Springer Science+Business Media
Symptoms of hereditary periodic fever syndromes usually begin during childhood. Fewer than 10% of people develop symptoms after age 18 and in most cases feel well between attacks.
Drugs Mentioned In This Article
