periodic fever, familial, autosomal dominant

(redirected from TNF receptor-associated periodic syndrome)

periodic fever, familial, autosomal dominant

A hereditary periodic fever syndrome (OMIM:142680) characterised by recurrent fever, abdominal pain, localised tender skin lesions and myalgia. The main complication is amyloidosis, which occurs in 25% of cases.
 
Molecular pathology
Caused by defects of TNFRSF1A, which encodes a major receptor for TNF-alpha that activates NF-kappaB, mediates apoptosis and regulates inflammation.
References in periodicals archive ?
The TNF receptor-associated periodic syndrome (TRAPS): emerging concepts of an autoinflammatory disorder.
Their research in human and mouse cells suggests that blocking these molecules could reduce inflammation in TNF receptor-associated periodic syndrome (TRAPS) and possibly other inflammatory diseases.