Maria Giuseppina Brizi, MD; Mauro Galeazzi, MD; Orso Maria Lucherini, PhD; Luca Cantarini, MD, PhD; Rolando Cimaz, MD
Potential Conflicts of Interest: None disclosed.
Brizi MG, Galeazzi M, Lucherini OM, Cantarini L, Cimaz R. Successful Treatment of Tumor Necrosis Factor Receptor–Associated Periodic Syndrome With Canakinumab. Ann Intern Med. 2012;156:907-908. doi: 10.7326/0003-4819-156-12-201206190-00027
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Published: Ann Intern Med. 2012;156(12):907-908.
Background: Tumor necrosis factor (TNF) receptor–associated periodic syndrome (TRAPS) is characterized by prolonged fever episodes with periorbital edema, migratory erythematous plaques, myalgia, and serositis (1). Renal amyloidosis is the most serious long-term complication, with prevalence ranging from 14% to 25% (1). As the most common autoinflammatory disorder related to an autosomal dominant gene, TRAPS is caused by mutations in the TNFRSF1A gene, which encodes the 55-kDa receptor for TNF-α (2). These mutations negatively affect both gene expression and function (2, 3).
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