Disease Information for TRAPS/Tumor necrosis factor Receptor Periodic Syndrome

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS;Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant inherited condition of periodic fever and pain; Most patients are of northern European descent; The attacks manifest as fever and pain in the joints, abdomen, muscles, skin, or eyes, with variations across patients; An acute-phase response occurs during the attacks; Patients with TRAPS are at risk for AA amyloidosis, the most common targets being the kidneys and liver; Soluble TNFRSF1A is usually low between the attacks and may be normal during the attacks, when TNF levels are high; TNFRSF1A is found in abnormally high numbers on leukocyte cell membranes; TRAPS is the first condition for which naturally occurring mutations in a TNF receptor were found; the mutations affect the soluble TNFRSF1A gene in the 12p13 region; In some patients, the pathogenesis involves defective TNFRSF1A shedding from cell membranes in response to a given stimulus; Thus, TRAPS is a model for a novel pathogenic concept characterized by failure to shed a cytokine receptor; This review compares TRAPS to other inherited periodic febrile conditions, namely, familial Mediterranean fever, Muckle-Wells syndrome, cold urticaria, and hyper-IgD syndrome; The place of TRAPS relative to other intermittent systemic joint diseases is discussed; Colchicine may prevent expected Amyloidosis but neither relieves nor prevents the attacks, whereas oral glucocorticoid therapy is effective when used in dosages greater than 20 mg/day(PUBMED)

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