title: PFAPA syndrome: a review on treatment and outcome.
abstract: 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 proven so far. Tonsillectomy is an option for selected patients. Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood.
authors.affiliation: Pediatric Department of Southern Switzerland, Ospedale Regionale di Bellinzona e Valli, Bellinzona, 6500, Switzerland. federica.vanoni@eoc.ch.
authors.forename: Federica
authors.identifier: http://orcid.org/0000-0003-1381-4647
authors.initials: F
authors.lastname: Vanoni
authors.affiliation: Unité Romande d'Immuno-rhumatologie Pédiatrique (URIRP), Département Femme-Mère-Enfant (DFME), CHUV, University of Lausanne, Rue du Bugnon 46, Lausanne, 1011, Switzerland.
authors.forename: Katerina
authors.initials: K
authors.lastname: Theodoropoulou
authors.affiliation: Unité Romande d'Immuno-rhumatologie Pédiatrique (URIRP), Département Médico - Chirurgical de Pédiatrie (DFME), CHUV, University of Lausanne, Rue du Bugnon 46, Lausanne, 1011, Switzerland.
authors.forename: Michaël
authors.initials: M
authors.lastname: Hofer
mesh_terms: D000293:Adolescent; D002648:Child; D003078:Colchicine / Q000627:therapeutic use*; D019468:Disease Management; D005334:Fever / Q000628:therapy*; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008199:Lymphadenitis / Q000628:therapy*; D009333:Neck; D010507:Periodicity; D010612:Pharyngitis / Q000628:therapy*; D013281:Stomatitis, Aphthous / Q000628:therapy*; D013577:Syndrome; D014068:Tonsillectomy; D050257:Tubulin Modulators / Q000627:therapeutic use*
chemical_list: D005938:Glucocorticoids; D050257:Tubulin Modulators; D003078:Colchicine
keywords: Colchicine; Glucocorticoids; PFAPA outcome; PFAPA treatment; Tonsillectomy