Produção Nacional
THE EFFICACY OF CANAKINUMAB IN THE TREATMENT OF TUMOR NECROSIS FACTOR (TNF) ALPHA RECEPTOR-ASSOCIATED PERIODIC SYNDROME (TRAPS)
2019; Linguagem: Inglês
10.5151/sbr2019-280
ISSN2357-7282
AutoresÉrica Naomi Naka Matos, Milena Foizer Leite, Carolina Yume Arazawa, QUEROLAI GOMES GADELHA, RENATO FURTADO TAVARES, LEONARDO PEREIRA WON MUHLEN,
Tópico(s)Chronic Lymphocytic Leukemia Research
ResumoTNF alpha receptor-associated periodic syndrome (TRAPS) is a rare autoinflammatory syndrome (AIS), of unknown prevalence and onset of symptoms during early infancy and childhood. It’s caused by at least 93 mutations described so far, of the gene TNFRSF1A and its inheritance is autosomal dominant. This nosologic entity presents with longer febrile periods, when compared to other AIS, abdominal pain, myalgia, erythematous/urticarial rash, conjunctivitis, uveitis, headache, arthritis and pleurisy. The diagnosis is clinical and laboratorial, once excluded the autoimmune and infectious diseases. Sequencing the TNFRSF1A gene in DNA genome demonstrates mutations in 32-50% of the cases analyzed. The present study hereby describes a child affected by TRAPS, who presented considerable improvement after using canakinumab, a fully humanized monoclonal antibody of the class IgG1, with strong affinity to IL-1β.
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