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Arthritis & rheumatology (Hoboken, N.J.) DOI:10.1002/art.38542

A121: in search of infectious triggers of periodic Fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Publication TypeJournal Article
Year of Publication2014
AuthorsFreeman, S, Bhatt, A, Pedamallu, C, King, S, Duke, F, Jung, J, Lawton, M, Anderson, E, Fuhlbrigge, RC, Kenna, M, Licameli, G, Meyerson, M, Dedeoglu, F
JournalArthritis & rheumatology (Hoboken, N.J.)
Volume66 Suppl 11
Date Published2014/03/01

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most prevalent pediatric autoinflammatory fever disorder. As there is no known genetic cause or confirmatory test, diagnosis of PFAPA is challenging. Clockwork interval between episodes is a characteristic feature, which aids in diagnosis. The true prevalence and the etiology of PFAPA are not known, but genetic factors leading to immune dysregulation as well as infectious agents have been suggested as causative factors. Oral corticosteroids abort the episodes in majority of patients, and in recent years, tonsillectomy has been shown to be effective in inducing remission. Our center has a significant success rate with tonsillectomy in PFAPA patients. Using unbiased next-generation sequencing, we investigated the presence of potential infectious agents and gene expression signatures in tonsils from patients with PFAPA, chronic tonsillitis and obstructive sleep apnea (OSA).