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dc.contributor.authorMagnotti, F.
dc.contributor.authorVitale, A.
dc.contributor.authorRigante, D.
dc.contributor.authorLucherini, O. M.
dc.contributor.authorCimaz, R.
dc.contributor.authorMuscari, I.
dc.contributor.authorFaria, Atila Granados Afonso de [UNIFESP]
dc.contributor.authorFrediani, B.
dc.contributor.authorGaleazzi, M.
dc.contributor.authorCantarini, L.
dc.identifier.citationClinical And Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 31, n. 3, p. S141-S149, 2013.
dc.description.abstractTumour necrosis factor-receptor associated periodic syndrome (TRAPS) is a rare autosomal dominant autoinflammatory disorder characterised by recurrent episodes of long-lasting fever and inflammation in different regions of the body, as musculo-skeletal system, skin, gastrointestinal tube, serosal membranes and eye. Inflammatory attacks usually start in paediatric age with initial corticosteroid-responsiveness. Most reported cases of TRAPS involve patients of European ancestry and diagnosis can be formulated by the combination of genetic analysis and a compatible phenotype. Its prognosis is strictly dependent on the appearance of amyloidosis, secondary to uncontrolled relapsing inflammation. Thanks to a better understanding of its pathogenesis, the disease is now managed with anti-interleukin (IL)-1 antagonists, rather than corticosteroids or tumour necrosis factor (TNF) inhibitors. The aim of this review is to describe the current understanding and advances of TRAPS genetic basis, pathogenesis and management options by integrating the most recent data in the medical literature.en
dc.publisherClinical & Exper Rheumatology
dc.relation.ispartofClinical And Experimental Rheumatology
dc.rightsAcesso restrito
dc.subjecttumour necrosis factor receptor-associated periodic syndrome (TRAPS)en
dc.subjectautoinflammatory disordersen
dc.subjectTNFRSF1A geneen
dc.subjectinterleukin (IL)-1 betaen
dc.titleThe most recent advances in pathophysiology and management of tumour necrosis factor receptor associated periodic syndrome (TRAPS): personal experience and literature reviewen
dc.contributor.institutionUniv Siena
dc.contributor.institutionUniv Cattolica Sacro Cuore
dc.contributor.institutionAnna Meyer Childrens Hosp
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.description.affiliationUniv Siena, Rheumatol Unit, Policlin Le Scotte, Res Ctr Syst Autoimmune & Autoinflammatory Dis, I-53100 Siena, Italy
dc.description.affiliationUniv Cattolica Sacro Cuore, Inst Paediat, Rome, Italy
dc.description.affiliationAnna Meyer Childrens Hosp, Rheumatol Unit, Dept Paediat, Florence, Italy
dc.description.affiliationUniv Cattolica Sacro Cuore, Rome, Italy
dc.description.affiliationUniv Fed Sao Paulo, Dept Med, Rheumatol Div, UNIFESP, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Med, Rheumatol Div, UNIFESP, Sao Paulo, Brazil
dc.description.sourceWeb of Science

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