title: Successful treatment of a patient with tumor necrosis factor receptor-associated periodic syndrome using a half-dose of etanercept.
abstract: TNF receptor-associated periodic syndrome (TRAPS) is caused by mutations of TNFRSF1A gene and characterized by recurrent febrile episodes of prolonged duration and initial good response to steroids. Etanercept, a TNF blocker, has been used as a putative molecular-targeted agent for TRAPS, with some patients showing limited efficacy. Here, we report a patient with TRAPS who recovered from steroid dependency by etanercept and kept remission with a reduced dose of etanercept. The pathophysiology of TRAPS still remains to be elucidated and several hypotheses have been proposed. In the most recent hypothesis, the concerted action of wild-type and mutant TNF receptors plays an important role in provoking enhanced inflammation in TRAPS. The excellent response to etanercept in our patient suggested that there is heterogeneity in TRAPS patients in terms of the contribution of normal TNF signaling to autoinflammation.
authors: Department of Pediatrics, University of Occupational and Environmental Health School of Medicine, Kitakyushu, Japan. kkusuhar@med.uoeh-u.ac.jp
authors: Koichi
authors: K
authors: Kusuhara
authors: Takayuki
authors: T
authors: Hoshina
authors: Mitsumasa
authors: M
authors: Saito
authors: Masataka
authors: M
authors: Ishimura
authors: Hirosuke
authors: H
authors: Inoue
authors: Takahiko
authors: T
authors: Horiuchi
authors: Tetsuji
authors: T
authors: Sato
authors: Toshiro
authors: T
authors: Hara
mesh_terms: D000293:Adolescent; D000068800:Etanercept; D005260:Female; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000097:blood / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D007074:Immunoglobulin G / Q000008:administration & dosage*; D010375:Pedigree; D018124:Receptors, Tumor Necrosis Factor / Q000008:administration & dosage / Q000037:antagonists & inhibitors*; D012074:Remission Induction
chemical_list: D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D000068800:Etanercept