The PFAPA syndrome: review on diagnosis and management

Introduction and purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome) is the most common periodic fever condition in child age. Discussed syndrome is generally a self-limited condition, but it can significantly decrease the quality of child’s life. It can be also challenge for parents, as well as medical professionals. Knowledge of the syndrome is very important among many specialists because early detection is the basis of the correct differentia and proper treatment. Many researches has been made to explain the genetic and immunologic elements which play a significant role in the pathogenesis of PFAPA, but it is not fully undestood. This review will focus on current knowledge regarding the pathogenesis of PFAPA, as well as surgical and conservative treatment strategies. Material and methods: This review was based on available data collected in the PubMed database and published between 2013 and 2022. The study was conducted by reviewing key words as follows: "PFAPA","treatment", "diagnosis","tonsillectomy". Results: The diagnosis of PFAPA syndrome consists of a typical clinical presentation, but sometimes diagnosis of this syndrome can be problematic. Some clinical criteria can be useful. We have vary of treatment arms depends on clinical targets. Depite that PFAPA is self limiting syndrome which can dissappear in later years of children’s life.   Conclusions: PFAPA syndrom is rare disease, but is very important to put emphasis on knowledge about it. Early diagnosis of this syndrome can make physicians send patient to specialized ward, and it will be possible to start proper and efficient treatment. It is a question of the future that we will get more patophysiologic knowledge about this disease