Pediatric fever, aphthous stomatitis, pharyngitis and adenitis
ZHANG Zhi-yong
ZHAO Xiao-dong
Abstract：Periodic fever-aphthous stomatitis-pharyngitis-adenitis (PFAPA) syndrome is the most frequent cause of recurrent fevers in childhood and presents with the cardinal symptoms of periodic fever,aphthous stomatitis,pharyngitis,and adenitis typically before 5 years old.The pathogenesis of PFAPA is still unknown and theories about the pathogenesis of PFAPA include faulty innate immunologic response with dysregulated T-cell activation.The potential hereditary nature of PFAPA is still disputed and the Mediterranean fever (MEFV) gene mutation,implicated in familial Mediterranean fever (FMF),has a possible association with PFAPA.It appears to modify disease severity.Diagnostic criteria include the traditional clinical signs,as well as the following biomarkers:elevated C-reactive protein,vitamin D,CD64,C-X-C motif ligand 10(CXCL10) and other nonspecific inflammatory mediators.Because PFAPA is self-limited and benign,there is no certain treatment model.Treatment for PFAPA includes a single dose of corticosteroids,tonsillectomy,and most recently,interleukin 1 inhibitors.Treatment options must be specific to the patient.More large cohort studies are needed to explore the inhereditary nature and specific biomarkers of PFAPA,to standardize the diagnosis and treatment process in order to improve the life quality of the children.
Keywords：periodic fever-aphthous stomatitis-pharyngitis-adenitisautoinflammatory disorderprimary immunodeficiency diseaseperiodic fever syndrome
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 14-17 )