The Enigma of Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome

Susanna Esposito, MD; Sonia Bianchini, MD; Miriam Fattizzo, MD; Elena Baggi, MD; Paola Marchisio, MD; Donato Rigante, MD


Pediatr Infect Dis J. 2014;33(6):650-652. 

In This Article

Treatment of PFAPA Syndrome

There is no consensus concerning the best remedy for PFAPA syndrome. The main therapies are essentially symptomatic and consist of single doses of corticosteroids: prednisone (1–2 mg/kg) or betamethasone (0.1–0.2 mg/kg) can abolish a febrile attack within a few hours. Kyvsgaard et al[19] observed that the oral administration of prednisolone controlled febrile episodes in 87.5% of patients, although it was also associated with shorter interfebrile periods. Feder and Salazar[20] found that cimetidine (150 mg twice a day for 6–12 months) interrupted fever attacks in only 27% of patients and that the prophylactic use of colchicine (0.5–1 mg/d) led to partial remissions with longer intervals between attacks. A meta-analysis of 14 studies published between 1999 and 2009 by Peridis et al[21] revealed that antibiotics were ineffective; that surgical therapy (tonsillectomy with or without adenoidectomy) was more effective than antibiotics and cimetidine prophylaxis and that there was no statistically significant difference between treatment with corticosteroids and surgery, although they concluded that tonsillectomy was the most effective intervention for the long-term resolution of PFAPA syndrome as the use of corticosteroids did not prevent further febrile cycles and also shortened interfebrile intervals.