title: Advances in the diagnosis and treatment of tumor necrosis factor receptor-associated periodic syndrome.
abstract: Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare autosomal dominant disease included in the group of autoinflammatory syndromes. It is characterized by recurrent episodes of fever and inflammation in different regions of the body. The main clinical manifestations are myalgia, migratory erythematous rash, periorbital edema, and abdominal pain. The diagnosis is reached using gene analysis and prognosis depends on the appearance of amyloidosis secondary to the recurrent episodes of inflammation. Tumor necrosis factor inhibitors and corticosteroids are the most widely used treatments. In recent years, significant advances have been made in the diagnosis and treatment of TRAPS, thanks to a better understanding of its pathogenesis. Dermatologists must be aware that the skin manifestations of TRAPS are particularly important, as they are often diagnostic.
authors.affiliation: Departamento de Dermatología, Clínica Universidad de Navarra, Pamplona, Spain. laguado@unav.es
authors.forename: L
authors.initials: L
authors.lastname: Aguado-Gil
authors.forename: I
authors.initials: I
authors.lastname: Irarrazaval-Armendáriz
authors.forename: M
authors.initials: M
authors.lastname: Pretel-Irazabal
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D017668:Age of Onset; D000686:Amyloidosis / Q000209:etiology / Q000517:prevention & control; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D015897:Comorbidity; D004252:DNA Mutational Analysis; D000068800:Etanercept; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D009103:Multiple Sclerosis / Q000235:genetics; D010493:Pericarditis / Q000209:etiology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000037:antagonists & inhibitors / Q000235:genetics*; D012008:Recurrence; D063189:Symptom Assessment; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D000305:Adrenal Cortex Hormones; D061067:Antibodies, Monoclonal, Humanized; D007074:Immunoglobulin G; D053590:Interleukin 1 Receptor Antagonist Protein; D018124:Receptors, Tumor Necrosis Factor; D047888:Receptors, Tumor Necrosis Factor, Type I; C506235:TNFRSF1A protein, human; D014409:Tumor Necrosis Factor-alpha; C502936:tocilizumab; D000068800:Etanercept; D003078:Colchicine
keywords: Autoinflammatory diseases; Enfermedades autoinflamatorias; Receptor del factor de necrosis tumoral; Syndrome; Síndrome; Síndrome periódico asociado al receptor del factor de necrosis tumoral; Tumor necrosis factor receptor; Tumor necrosis factor receptor-associated periodic syndrome