Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease
Joint Authors
Cantarini, Luca
Lopalco, Giuseppe
Galeazzi, Mauro
Cattalini, Marco
Soliani, Martina
Rigante, Donato
Iannone, Florenzo
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-08-18
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
Autoinflammatory diseases are caused by inflammasome dysregulation leading to overproduction of proinflammatory cytokines and a pathological delay in the inflammation switching off.
The progress of cellular biology has partially clarified pathogenic mechanisms behind monogenic autoinflammatory diseases, whereas little is known about the polygenic ones.
Although the genetic susceptibility of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) syndrome is still obscure, the presence of overlapping symptoms with monogenic periodic fevers, the recurrence in family members, the important role played by dysregulated interleukin- (IL-) 1β secretion during flares, the overexpression of inflammasome-associated genes during attacks, and, last but not least, the therapeutic efficacy of IL-1β blockade strongly indicate a potential genetic involvement in its pathogenesis, probably linked with environmental factors.
PFAPA syndrome has a typical inception in the pediatric age, but a delayed onset during adulthood has been described as well.
Treatments required as well as effectiveness of tonsillectomy remain controversial, even if the disease seems to have a self-limited course mostly in children.
The purpose of this review is to provide an overview of this complex polygenic/multifactorial autoinflammatory disorder in which the innate immune system undoubtedly plays a basic role.
American Psychological Association (APA)
Cattalini, Marco& Soliani, Martina& Rigante, Donato& Lopalco, Giuseppe& Iannone, Florenzo& Galeazzi, Mauro…[et al.]. 2015. Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease. Mediators of Inflammation،Vol. 2015, no. 2015, pp.1-11.
https://search.emarefa.net/detail/BIM-1072431
Modern Language Association (MLA)
Cattalini, Marco…[et al.]. Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease. Mediators of Inflammation No. 2015 (2015), pp.1-11.
https://search.emarefa.net/detail/BIM-1072431
American Medical Association (AMA)
Cattalini, Marco& Soliani, Martina& Rigante, Donato& Lopalco, Giuseppe& Iannone, Florenzo& Galeazzi, Mauro…[et al.]. Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease. Mediators of Inflammation. 2015. Vol. 2015, no. 2015, pp.1-11.
https://search.emarefa.net/detail/BIM-1072431
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1072431