The most recent advances in pathophysiology and management of tumor necrosis factor receptor-associated periodic syndrome (TRAPS): personal experience and review of literature

Donato Rigante, F Magnotti, A Vitale, Om Lucherini, R Cimaz, I Muscari, A Granados Afonso De Faria, B Frediani, M Galeazzi, L. Cantarini

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Tumour 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 the pediatric 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.
Lingua originaleEnglish
pagine (da-a)141-149
Numero di pagine9
RivistaCLINICAL AND EXPERIMENTAL RHEUMATOLOGY
Volume2013
Stato di pubblicazionePubblicato - 2013

Keywords

  • TRAPS

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