The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been...

Introduction Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is an autoinflammatory disorder of childhood and little is known about the underlying etiology, pathogenesis or the reason behind the success of tonsillectomy. Numerous immune cell types are present in the tonsillar microenvironment, each of which may contribute to the pattern of symptoms observed in PFA...

Introduction PFAPA syndrome is the most common cause of periodic fevers in children. In the clinical setting of children with periodic fever, there are a minority of patients in which to differentiate between PFAPA and monogenic periodic fevers is not immediate. The Gaslini score would provide a useful tool in this setting, calculating the probability to carry a mutation for monogenic periodic ...

Introduction The PFAPA syndrome is a non-mendelian autoinflammatory disease of unknown aetiology characterized by Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis. In typical cases, attacks begin before the age of five and occur every 2-8 weeks, often with striking periodicity. We have previously profiled the blood cells and serum cytokine levels in a cohort study of pati...

OBJECTIVE To identify a set of variables that could discriminate patients with adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome from subjects with fever of unknown origin (FUO). METHODS We enrolled 74 adults diagnosed with PFAPA syndrome according to the currently used pediatric diagnostic criteria and 62 additional patients with FUO. After ...

Як свідчать дані літератури, синдром РFАРА – періодична гарячка з тонзилофарингітом, афтозним стоматитом і шийним лімфаденітом вперше описали вчені G.S. Marshall et al. у 1987 р. Цей є поширеним автозапальним розладом дітей, але трапляється в дорослих, багатьох частинах світу. Його етіологія патогенез ще не з’ясовані. Триває пошук удосконалення діагностичних критеріїв.
 На сьогодні викорис...

Background The pathogenesis of PFAPA syndrome is unknown. According to Stojanov et al. cytokine profile in PFAPA syndrome suggests Th1 mediated inflammatory process resulting in continuous inflammation and reduced Th2 anti-inflammatory response. Interferon γ might be responsible for suppressing the production of IL-4 and IL10. Hung et al. showed suppressive effects of ketotifen on the expressio...

Introduction Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical adenitis and leukocytosis. Little is known about the true incidence, natural course, pathogenesis, and appropriate therapy in this recently described syndrome. While the mechanism is ...

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is a non-hereditary autoinflammatory disease, characterized by relatively regular recurrence of febrile episodes of 3-6 days duration, accompanied by aphthous stomatitis, pharyngitis/tonsillitis, and/or cervical adenititis. It is considered to be the most common periodic fever syndrome in Japan. Although no responsib...

Purpose PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis) is an idiopathic autoinflammatory disease with the first manifestation before 5 years of age. Fever attacks lasting for 3-6 days have individual periodicity within about 3-8 week intervals. They are accompanied by individual combination of other symptoms among which culture-negative pharyngitis/tonsillitis, o...