Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed before 5 years, though reports regarding adulthood are increasing. The pathogenesis of the syndrome is no...

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Bibliographic Details
Published in:Clinical rheumatology 2019-01, Vol.38 (1), p.11-17
Main Authors: Gaggiano, Carla, Rigante, Donato, Sota, Jurgen, Grosso, Salvatore, Cantarini, Luca
Format: Article
Language:English
Subjects:
Adenitis
Anti-inflammatory agents
Aphthous stomatitis
Children
Cimetidine
Colchicine
Corticosteroids
Fever
Inflammation
Interleukin 1
Medicine
Medicine & Public Health
Patients
Pediatrics
Pharyngitis
Review Article
Rheumatology
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Summary:Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed before 5 years, though reports regarding adulthood are increasing. The pathogenesis of the syndrome is not completely understood, but a multifactorial origin, probably based on a polygenic pattern of susceptibility, is the most probable rational pathogenetic hypothesis. Treatment of PFAPA syndrome relies on the administration of low-dose corticosteroids, which promptly abort flares but cannot prevent subsequent disease episodes over time. Tonsillectomy with or without adenoidectomy has proved to be successful in some pediatric patients, as proven by different studies. On the other hand, colchicine, cimetidine, nonsteroidal anti-inflammatory drugs, and interleukin-1 inhibitors have shown efficacy, which require further definite confirmations. This review is aimed at summarizing all the recent evidence about treatment options available for PFAPA syndrome both in pediatric and adult patients.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-018-4361-2