Understanding Periodic Fever Syndromes
Periodic fever syndromes are a group of rare, often inherited conditions; there may be a family history of similar episodes. The episodes of fever are not caused by infection and they are not due to an autoimmune disease such as systemic lupus erythematosus. Periodic fever syndromes typically involve episodes of unregulated inflammation, which is why they are also known as autoinflammatory conditions.
Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
PFAPA is probably the most common disorder. It often appears in early childhood (between the ages of 2 and 5). Children have recurrent episodes of fever, mouth sores, sore throat, and swollen lymph nodes in the neck. There tend to be regular intervals between episodes. There are no long-term complications associated with PFAPA, and treatment is recommended to minimize or eliminate symptoms and allow a return to regular activities. PFAPA may last for several years, but it often goes away on its own during the second decade of life.
Familial Mediterranean Fever (FMF)
This genetic disease mostly affects people of Mediterranean and Middle Eastern descent. It causes recurrent fevers along with pain and swelling in the abdomen, chest, or joints. Painful, severe joint swelling can make it hard to walk. It typically affects children before the age of 10. Left untreated, FMF may result in a dangerous buildup of proteins, called amyloidosis, that may lead to organ failure.
Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
This condition causes intermittent fevers plus a painful rash, chills, and muscle pain. Onset is often in childhood but may be delayed until mid-adulthood. In addition to symptoms common to other periodic fever syndromes, people with TRAPS often have associated eye symptoms including conjunctivitis and swelling around the eye. Amyloidosis may occur in some people.
Hyperimmunoglobulin D Syndrome (HIDS)
This rare genetic condition is also known as Mevalonate Kinase-Associated Periodic Fever Syndrome. Symptoms usually start in the first year of life. It typically begins with an abrupt onset of high fever (up to 104). Associated symptoms may include skin rash, abdominal pain, vomiting, diarrhea, joint pain, and swollen neck glands.