Matches in SemOpenAlex for { <https://semopenalex.org/work/W2898082236> ?p ?o ?g. }
- W2898082236 endingPage "58" @default.
- W2898082236 startingPage "27" @default.
- W2898082236 abstract "Recurrent fever syndromes (RFS) are autoinflammatory disorders characterized by self-limited recurrent episodes of fever and systemic inflammation, associated with inflammatory manifestations. Five clinical entities can be considered as the prototypical RFS: familial Mediterranean fever (FMF), tumour necrosis factor (TNF), receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), cryopyrin-associated periodic syndromes (CAPS), and periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA). Genetic testing plays a key role in establishing their diagnosis. In addition, recent development of next-generation sequencing technologies has led to the rapid identification of a number of additional genes responsible for very rare RFS. Since RFS affect multiple organs with potentially severe complications, management of patients is complex and warrants a multidisciplinary approach. The main therapeutic options encompass nonspecific anti-inflammatory approaches, such as corticosteroids and non-steroidal anti-inflammatory drugs, as well as biological agents. The study of the pathophysiology of RFS led to the discovery of multiple receptors of the innate immune system recognizing pathogens and endogenous danger signals. Although a number of pathogenic processes have been demonstrated as underlying these disorders, dysregulation of the interleukin-1β (IL-1β) signalling pathway was found to play a key role in most RFS. This catalysed major advances in the development of targeted biologic therapies, particularly IL-1 blocking treatments. Nevertheless, some patients will require more tailored therapeutic approaches." @default.
- W2898082236 created "2018-10-26" @default.
- W2898082236 creator A5004921372 @default.
- W2898082236 date "2018-10-17" @default.
- W2898082236 modified "2023-09-23" @default.
- W2898082236 title "Recurrent Fever Syndromes" @default.
- W2898082236 cites W1524848610 @default.
- W2898082236 cites W1542410645 @default.
- W2898082236 cites W1550548733 @default.
- W2898082236 cites W1581139251 @default.
- W2898082236 cites W1596257295 @default.
- W2898082236 cites W1597243980 @default.
- W2898082236 cites W1780500931 @default.
- W2898082236 cites W1789511037 @default.
- W2898082236 cites W1880023855 @default.
- W2898082236 cites W1895520487 @default.
- W2898082236 cites W1901914253 @default.
- W2898082236 cites W1951926033 @default.
- W2898082236 cites W1964034086 @default.
- W2898082236 cites W1965668934 @default.
- W2898082236 cites W1966351563 @default.
- W2898082236 cites W1968047575 @default.
- W2898082236 cites W1968057560 @default.
- W2898082236 cites W1969019918 @default.
- W2898082236 cites W1970338512 @default.
- W2898082236 cites W1972483153 @default.
- W2898082236 cites W1973929401 @default.
- W2898082236 cites W1975484759 @default.
- W2898082236 cites W1976109352 @default.
- W2898082236 cites W1977884267 @default.
- W2898082236 cites W1978462186 @default.
- W2898082236 cites W1980442164 @default.
- W2898082236 cites W1981093010 @default.
- W2898082236 cites W1984422262 @default.
- W2898082236 cites W1985383819 @default.
- W2898082236 cites W1988473650 @default.
- W2898082236 cites W1992002214 @default.
- W2898082236 cites W1993134828 @default.
- W2898082236 cites W1994115980 @default.
- W2898082236 cites W1996363381 @default.
- W2898082236 cites W1996830451 @default.
- W2898082236 cites W1997637712 @default.
- W2898082236 cites W1997702653 @default.
- W2898082236 cites W1998145026 @default.
- W2898082236 cites W1999677397 @default.
- W2898082236 cites W2001444906 @default.
- W2898082236 cites W2001747858 @default.
- W2898082236 cites W2002056653 @default.
- W2898082236 cites W2004510613 @default.
- W2898082236 cites W2006813791 @default.
- W2898082236 cites W2009004909 @default.
- W2898082236 cites W2009268420 @default.
- W2898082236 cites W2009800174 @default.
- W2898082236 cites W2010048822 @default.
- W2898082236 cites W2012883414 @default.
- W2898082236 cites W2013606896 @default.
- W2898082236 cites W2015310325 @default.
- W2898082236 cites W2016935631 @default.
- W2898082236 cites W2017237017 @default.
- W2898082236 cites W2017500465 @default.
- W2898082236 cites W2017867878 @default.
- W2898082236 cites W2018915861 @default.
- W2898082236 cites W2019700846 @default.
- W2898082236 cites W2020908160 @default.
- W2898082236 cites W2024378513 @default.
- W2898082236 cites W2024448924 @default.
- W2898082236 cites W2028368428 @default.
- W2898082236 cites W2029289043 @default.
- W2898082236 cites W2032140602 @default.
- W2898082236 cites W2033023137 @default.
- W2898082236 cites W2034391829 @default.
- W2898082236 cites W2035027484 @default.
- W2898082236 cites W2035101626 @default.
- W2898082236 cites W2035113730 @default.
- W2898082236 cites W2037496259 @default.
- W2898082236 cites W2038636068 @default.
- W2898082236 cites W2041084308 @default.
- W2898082236 cites W2043710737 @default.
- W2898082236 cites W2045015441 @default.
- W2898082236 cites W2048032544 @default.
- W2898082236 cites W2048491075 @default.
- W2898082236 cites W2048791692 @default.
- W2898082236 cites W2048819076 @default.
- W2898082236 cites W2048982572 @default.
- W2898082236 cites W2052847333 @default.
- W2898082236 cites W2053114531 @default.
- W2898082236 cites W2055177114 @default.
- W2898082236 cites W2058243697 @default.
- W2898082236 cites W2058590329 @default.
- W2898082236 cites W2062059653 @default.
- W2898082236 cites W2062641743 @default.
- W2898082236 cites W2062946728 @default.
- W2898082236 cites W2065239649 @default.
- W2898082236 cites W2066635545 @default.
- W2898082236 cites W2072022640 @default.
- W2898082236 cites W2074547696 @default.
- W2898082236 cites W2075871827 @default.
- W2898082236 cites W2076963123 @default.