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

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

Author Magnotti, F. Google Scholar
Vitale, A. Google Scholar
Rigante, D. Google Scholar
Lucherini, O. M. Google Scholar
Cimaz, R. Google Scholar
Muscari, I. Google Scholar
Faria, Atila Granados Afonso de Autor UNIFESP Google Scholar
Frediani, B. Google Scholar
Galeazzi, M. Google Scholar
Cantarini, L. Google Scholar
Institution Univ Siena
Univ Cattolica Sacro Cuore
Anna Meyer Childrens Hosp
Universidade Federal de São Paulo (UNIFESP)
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 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.
Keywords tumour necrosis factor receptor-associated periodic syndrome (TRAPS)
autoinflammatory disorders
amyloidosis
TNFRSF1A gene
interleukin (IL)-1 beta
Language English
Date 2013-05-01
Published in Clinical And Experimental Rheumatology. Pisa: Clinical & Exper Rheumatology, v. 31, n. 3, p. S141-S149, 2013.
ISSN 0392-856X (Sherpa/Romeo, impact factor)
Publisher Clinical & Exper Rheumatology
Extent S141-S149
Origin http://www.clinexprheumatol.org/abstract.asp?a=6886
Access rights Closed access
Type Review
Web of Science ID WOS:000326356700028
URI http://repositorio.unifesp.br/11600/43336

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