TY - JOUR
T1 - The most recent advances in pathophysiology and management of tumour necrosis factor receptor-associated periodic syndrome (TRAPS): Personal experience and literature review
AU - Magnotti, Flora
AU - Vitale, Antonio
AU - Rigante, Donato
AU - Lucherini, Orso Maria
AU - Cimaz, Rolando
AU - Muscari, Isabella
AU - De Faria, Atila Granados Afonso
AU - Frediani, Frediani
AU - Galeazzi, Mauro
AU - Cantarini, Luca
PY - 2013
Y1 - 2013
N2 - 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 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. © Clinical and Experimental Rheumatology 2013.
AB - 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 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. © Clinical and Experimental Rheumatology 2013.
KW - Amyloidosis
KW - Autoinflammatory disorders
KW - Interleukin (IL)-1β
KW - TNFRSF1A gene
KW - Tumour necrosis factor receptor-associated periodic syndrome (TRAPS)
KW - Amyloidosis
KW - Autoinflammatory disorders
KW - Interleukin (IL)-1β
KW - TNFRSF1A gene
KW - Tumour necrosis factor receptor-associated periodic syndrome (TRAPS)
UR - http://hdl.handle.net/10807/191865
M3 - Article
SN - 0392-856X
VL - 31
SP - S141-S149
JO - Clinical and Experimental Rheumatology
JF - Clinical and Experimental Rheumatology
ER -