Periodic fever, aphthous stomatitis, pharyngitis and adenitis
PFAPA (Periodic fever - aphthous stomatitis- pharyngitis - adenopathy) syndrome is an auto inflammatory syndrome characterized by recurrent febrile episodes associated with aphthous stomatitis, pharyngitis and cervical adenitis
Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a medical condition, typically occurring in young children, in which high fever occurs periodically at intervals of about 3–5 weeks, frequently accompanied by aphthous-like ulcers, pharyngitis and cervical adenitis (cervical lymphadenopathy). The syndrome was described in 1987 and named two years later.
Signs and symptoms
The key symptoms of PFAPA are those in its name: periodic high fever at intervals of about 3–5 weeks, as well as aphthous ulcers, pharyngitis and adenitis. In between episodes, and even during the episodes, the children appear healthy. At least 6 months of episodes.
Cause
The cause of PFAPA is unknown. It is frequently discussed together with other periodic fever syndromes.
Possible causes include primarily genetic factors or it may be due to an initial infection.
The condition appears to be the result of a disturbance of innate immunity. The changes in the immune system are complex and include increased expression of complement related genes (C1QB, C2, SERPING1), interleukin-1-related genes (interleukin-1β, interleukin 1 RN, CASP1, interleukin 18 RAP) and interferon induced (AIM2, IP-10/CXCL10) genes.
Begin with the source’s own compact description: “Periodic fever, aphthous stomatitis, pharyngitis and adenitis” is pFAPA (Periodic fever - aphthous stomatitis- pharyngitis - adenopathy) syndrome is an auto inflammatory syndrome characterized by recurrent febrile episodes associated with aphthous stomatitis, pharyngitis and cervical adenitis. The dossier treats that line as a proposition to test through Periodic, fever and aphthous, not as a finished interpretation.
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Named sources, stable identifiers and responsible institutions provide the strongest route from overview to verifiable evidence. The source revision retrieved here is dated May 16, 2026. The linked authority identifier is Q2044034. None of the 0 selected statements returned an explicit reference. The first chronological checks are 1987.
The absence of detail may reflect summary conventions rather than a lack of surviving documentation. The source lead contains qualifying language; that uncertainty should survive quotation, summary and reuse. Authority statements aid reconciliation but still require their own references, qualifiers and ranks to be checked.
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This entry incorporates text from “Periodic fever, aphthous stomatitis, pharyngitis and adenitis” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.