Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis

INTRODUCTION: The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a non-hereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. PFAPA does not cause long-lasting sequelae. An early diagnosis provides treatment possibilities for the patient and comfort to the family. MATERIAL AND METHODS: This study is a retrospective review of the medical records of patients diagnosed with PFAPA and admitted to our clinic from January 1999 to January 2010 (n = 31). RESULTS: The study population (n = 31) consisted of 21 males and ten females: 30 Caucasians and 1 Asian. Normal growth was seen in 30 patients. The median age at onset was 33 months. The mean duration of fever episodes was 4.45 days (95% confidence interval (CI): 3.92-4.98), and the mean duration of intervals between fever episodes was 29.66 days (95% CI: 25.31-34.01). Concomitantly with the fever, all patients had characteristic symptoms. All patients were asymptomatic in between their fever episodes. Prodromal symptoms were seen in 12 patients. Oral prednisolone was used in 24 patients and caused immediate fever reduction in 87.5%. A reduction in the duration of the asymptomatic interval after treatment was seen in 75.0%. Tonsillectomy was performed in 20 of the 31 patients causing cessation of fever episodes in 70%. Fever episodes continued in 15%, and the postoperative status remained unknown in the last 15%. Spontaneous resolution was seen in four patients. The diagnostic delay had a median duration of 28 months (range 2-160 months). CONCLUSION: The long diagnostic delay of PFAPA gives cause for concern and it indicates a need for greater awareness of the disease so that the diagnosis may be made earlier.