Successful Treatment of Tumor Necrosis Factor Receptor–Associated Periodic Syndrome With Canakinumab

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Letters19 June 2012Successful Treatment of Tumor Necrosis Factor Receptor–Associated Periodic Syndrome With CanakinumabMaria Giuseppina Brizi, MD, Mauro Galeazzi, MD, Orso Maria Lucherini, PhD, Luca Cantarini, MD, PhD, and Rolando Cimaz, MDMaria Giuseppina Brizi, MDFrom University of Siena, 53100 Siena, Italy, and University of Florence, 50139 Florence, Italy.Search for more papers by this author, Mauro Galeazzi, MDFrom University of Siena, 53100 Siena, Italy, and University of Florence, 50139 Florence, Italy.Search for more papers by this author, Orso Maria Lucherini, PhDFrom University of Siena, 53100 Siena, Italy, and University of Florence, 50139 Florence, Italy.Search for more papers by this author, Luca Cantarini, MD, PhDFrom University of Siena, 53100 Siena, Italy, and University of Florence, 50139 Florence, Italy.Search for more papers by this author, and Rolando Cimaz, MDFrom University of Siena, 53100 Siena, Italy, and University of Florence, 50139 Florence, Italy.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-156-12-201206190-00027 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail 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).Treating TRAPS is more challenging than treating other autoinflammatory syndromes because of its genetic heterogeneity and protean clinical phenotype. ...