Clinical and laboratory evaluation of cases with periodic fever aphthous stomatitis, pharyngitis and adenopathy syndrome M. Hizarcioglua, S. Asilsoya, G. D. Özeka, H. Agina, E. Kayserili, P. Güleza, H. Apa
Turkiye Klinikleri Tıp Bilimleri Dergisi 2008: 28: 648–652
Periodic Fever Aphthous Stomatitis, Pharyngitis and Adenopathy (PFAPA) syndrome is characterized by fever, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy. This syndrome is sporadic and has no specific laboratory findings. The aim of this study was to evaluate the clinical and laboratory findings of the patients with PFAPA syndrome and to determine the response to treatment. A retrospective analysis was conducted on the patients, who presented with periodic fever and were diagnosed as PFAPA syndrome between January 2005 and January 2008.
12 children (9 males, 3 females) with PFAPA syndrome between age 2 to 5 years were evaluated. Clinical findings were diagnosed at a mean age of 19.83 ± 11.51 months. Febrile attacks lasted for 3-6 days and they recurred every 4.16 ± 1.11 weeks. Other symptoms associated with high fever were pharyngitis (100%), cervical adenopathy (91%), aphthous stomatitis (66%) and exudative tonsillitis (58%). All patients were given oral prednisolone (1–2 mgr/kg/day) and their body temperature temporarily decreased within 2 to 8 h and did not increase up to the next cycle. The period between febrile episodes seemed to lengthen following steroid therapy; however the symptoms recurred. Three patients were given cimetidine. One of them had a longer period between febrile episodes, however the other two were not affected. Two patients who had undergone adeno-tonsillectomy did not have any further febrile episodes. They concluded that PFAPA syndrome can be easily diagnosed by detailed history, physical findings and observation of the patients who have periodic febrile episodes with tonsillo-pharyngitis. Early diagnosis will also prevent unnecessary antibiotic therapy in such patients.