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Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis,
Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric
Expression of Disease
#MMPMID26357457
Cattalini M
; Soliani M
; Rigante D
; Lopalco G
; Iannone F
; Galeazzi M
; Cantarini L
Mediators Inflamm
2015[]; 2015
(?): 570418
PMID26357457
show ga
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-) 1? secretion during
flares, the overexpression of inflammasome-associated genes during attacks, and,
last but not least, the therapeutic efficacy of IL-1? 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.
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