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How are Other Periodic Fever Syndromes different from FCAS?
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All Periodic Fever Syndromes share the fact that they all have periods of fevers and inflammation that subside, or disappear for periods of time, but are never resolved or cured. See each syndrome below for details about how these syndromes are different from FCAS, and the other CAPS disorders.
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Familial Mediterranean Fever (FMF)
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Familial Mediterranean Fever (FMF) has longer episodes of fever, a lot more problems than FCAS (peritonitis, pleursy, or even pericardial effusions), a different rash, arthritis-especially in the ankles, and abdominal pain. They can also develop amyloidosis. There is a genetic test for the FMF gene mutation (MEFV). The FMF genetic mutation has a protein pyrin that is similar to the pyrin domain for MWS, FCAS and NOMID/CINCA. FMF is much more common that FCAS, MWS, or NOMID/CINCA as well. People of Mediterranean decent are the most comon carriers of this hereditary periodic fever syndrome. Of note: There are many cases of NOMID/CINCA patients having had pericardial effusions as well, so testing for CAPS syndromes should be taken into consideration if someone has that problem occurs, and does not "fit" the FMF symptoms.
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Hyper IgD Syndrome (HIDS),
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Hyper IgD Syndrome (HIDS) also has longer episodes of flare ups than FCAS, a different rash, diarrhea, adenopathies and often, increased IGD levels (but these levels can go up and down with flare ups). NOMID/CINCA, MWS, and FCAS do not generally have elevated Ig D levels. (See links for HIDS.net link)
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TRAPS TNF-Receptor Associated Periodic Syndrome
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TRAPS (TNF-Tumor Necrosis Factor) Receptor Associated pPeriodic Fever Syndrome) has longer episodes of flares than FCAS, a different rash and periorbital edema (swelling around the eyes). There is a genetic test for TRAPS as well, and it is also known by other names, such as Familial Hibernian Fever (FHF).
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What about the Autoinflammatory syndromes, like Juvenile Idiopathic Arthritis (JIA)?
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People with JIA (Juvenile Idiopathic Arthritis) can have recurrent fevers, a rash, joint pain and joint deformities, can occasionally have pericardial effusions but there are some significant differences between JIA, an Autoimmune disease (the body attacks itself due to many other factors), and that of NOMID- a genetic autoinflammatory syndrome, which is caused by a mutation of a gene in the cryopyrin protein. With NOMID, the rash is different, and is present at birth, or early infancy, and the joints with NOMID patients can have non-specific joint pain and sometimes bony changes on X rays, along with enlarged knee-caps. These symptoms are NOT present with JIA. JIA patients usually have inflammation in their synovial joints, such as the shoulders or knees, with increased fluid levels in the joints from inflammation that caused JIA sufferers to have warm, swollen and stiff joints. Also, JIA patients do not have the multisystemic inflammatory involvement of the brain tissue, eyes, hearing, and other problems present in NOMID, or cold induction of symptoms seen with FCAS, or adolescent hearing loss and amyloidosis seen with MWS.
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PFAPA
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Periodic Fever, Aphthous stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) is also known as Marshall Syndrome. It is a chronic condition- often in in children under five, characterized by periodic episodes of high fever occurring every four weeks. The fever quickly rises to 103-106 degrees and stays high for three to six days. The child is healthy otherwise, with no runny nose or respiratory symptoms of illness. The syndrome may be accompanied by mouth sores, a red and inflamed throat, and swollen glands. Fevers resolve on their own spontaneously, but the periodic fevers may persist for several years but seems to have no significant health effects. This is a more common periodic fever syndrome, but physicians are often unaware of the syndrome. Some use of prednisone, or tonsillectomies have helped some patients, but much more research needs to be done to find answers for this syndrome. (See the links page for the PFAPA support group) Thanks Fran!
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