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

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.

journal_name

Clin Exp Rheumatol

journal_title

Clinical and experimental rheumatology

authors

Magnotti F,Vitale A,Rigante D,Lucherini OM,Cimaz R,Muscari I,Granados Afonso de Faria A,Frediani B,Galeazzi M,Cantarini L

subject

Has Abstract

pub_date

2013-05-01 00:00:00

pages

141-9

issue

3 Suppl 77

eissn

0392-856X

issn

1593-098X

pii

6886

journal_volume

31

pub_type

杂志文章,评审

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