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Joint Bone Spine
Volume 71, n° 4
pages 284-290 (juillet 2004)
Doi : 10.1016/j.jbspin.2003.10.008
Received : 22 August 2003 ;  accepted : 20 October 2003
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): definition, semiology, prognosis, pathogenesis, treatment, and place relative to other periodic joint diseases

Charles  Masson * ,  Virginie  Simon,  Emmanuel  Hoppé,  Paolo  Insalaco,  Idrissa  Cissé,  Maurice  Audran
Rheumatology Department, Angers Teaching Hospital, 49033 Angers cedex 1, France 

*Corresponding author.
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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 neither relieves nor prevents the attacks, whereas oral glucocorticoid therapy is effective when used in dosages greater than 20 mg/day. The pathogenic hypothesis involving defective TNFRSF1A shedding suggests that medications targeting TNF may be effective in TRAPS.

Mots clés  : TRAPS (TNF-receptor-associated periodic syndrome) ; Familial Mediterranean fever ; Muckle-Wells syndrome ; Familial cold urticaria ; Hyper-IgD syndrome.




© 2003  Elsevier SAS. All Rights Reserved.

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