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| TRAPS TNF-Receptor Associated Periodic Syndrome | |
TRAPS (TNF-Tumor Necrosis Factor) Receptor Associated Periodic Fever Syndrome is caused by a mutation of the TNFRSF1A gene. There is a genetic test for TRAPS as well, and it is also known by other names, such as Familial Hibernian Fever (FHF). TRAPS is considered to be the second most common known inherited periodic fever syndrome, with over 1,000 patients diagnosed worldwide. Still it is a very rare disease! Familial Mediterranean Fever is more common. TRAPS is inherited as an autosomal dominant syndrome, meaning that in most cases, you can inherit, or have a spontaneous mutation of the for TRAPS, and you will have symptoms. Most cases of TRAPS are inherited from an affected parent.
Most patients have their first attack or flare of TRAPS by the three years of age, but some patients do not start to have symptoms until they are in their twenties, or even later in life. Symptom flares can occur every 6 weeks, to a few times a year for many patients. Some patients may even have a few years between TRAPS flares.
TRAPS patients have longer episodes of flares that can last for three weeks or even longer, often with fevers that last for days. TRAPS presents with a rash that migrates all over the body, often with deep tissue pain under the areas of rash. Severe pain can follow the rash path on the limbs and body. TRAPS patients often have periorbital edema (swelling around the eyes) and conjunctivitis (redness of the eyes) during flares. TRAPS patients can also have joint pain, and even inflammation and pain in their mouth, throat and entire digestive tract, and even have their heart muscle inflammed.
CAPS patients can have conjunctivitis as well, but they do NOT generally have deep pain under a CAPS rash, and flares come and go,usually within 24 hours with FCAS, but a bit longer with other forms of CAPS. In TRAPS, the flares can last for many days or weeks.
The Genetics Home Reference article about TRAPS has more information about TRAPS
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