BACKGROUND: In children with recurrent episodes of fever, the cause may be the periodic fever syndrome (PFAPA-syndrome). The condition is not uncommon, and awareness of the syndrome is important for avoiding unnecessary investigations and treatment. The article presents an overview of the PFAPA-syndrome. MATERIAL AND METHODS: The overview was based on Pubmed and Medline searches and data from 22 children with PFAPA-syndrome diagnosed at Stavanger University Hospital. RESULTS AND INTERPRETATION: In children with PFAPA-syndrome the fever occurs regularly, appears abruptly and lasts for three to five days. Typical symptoms are cervical adenitis, tonsillitis/pharyngitis or aphthous stomatitis, often accompanied by headache, abdominal pain, nausea and reduced general condition. Of the 22 children, 17 were boys. The median age of debut was 12 months, median duration of fever four days, and median time between episodes 25 days. The most common symptoms were cervical adenitis (n = 18) and tonsillitis/pharyngitis (n = 16). During episodes, all children had high fever, reduced general condition, no proved infection but typical high levels of C-reactive protein. More than half of the children had been given antibiotics on at least five occasions before the diagnosis of PFAPA-syndrome. With a typical history and clinical investigation, the need for further investigations is limited. The diagnosis must be considered in children younger than five years of age with periodic fever without signs of airway infection. When PFAPA-syndrome is suspected, the child should be referred to a paediatrician. There is no evidence-based treatment for PFAPA-syndrome, but tonsillectomy is considered to have an effect.
BACKGROUND: In children with recurrent episodes of fever, the cause may be the periodic fever syndrome (PFAPA-syndrome). The condition is not uncommon, and awareness of the syndrome is important for avoiding unnecessary investigations and treatment. The article presents an overview of the PFAPA-syndrome. MATERIAL AND METHODS: The overview was based on Pubmed and Medline searches and data from 22 children with PFAPA-syndrome diagnosed at Stavanger University Hospital. RESULTS AND INTERPRETATION: In children with PFAPA-syndrome the fever occurs regularly, appears abruptly and lasts for three to five days. Typical symptoms are cervical adenitis, tonsillitis/pharyngitis or aphthous stomatitis, often accompanied by headache, abdominal pain, nausea and reduced general condition. Of the 22 children, 17 were boys. The median age of debut was 12 months, median duration of fever four days, and median time between episodes 25 days. The most common symptoms were cervical adenitis (n = 18) and tonsillitis/pharyngitis (n = 16). During episodes, all children had high fever, reduced general condition, no proved infection but typical high levels of C-reactive protein. More than half of the children had been given antibiotics on at least five occasions before the diagnosis of PFAPA-syndrome. With a typical history and clinical investigation, the need for further investigations is limited. The diagnosis must be considered in children younger than five years of age with periodic fever without signs of airway infection. When PFAPA-syndrome is suspected, the child should be referred to a paediatrician. There is no evidence-based treatment for PFAPA-syndrome, but tonsillectomy is considered to have an effect.