Periodic fever, aphthous stomatitis, pharyngitis and adenitis: PFAPA syndrome in Argentina

IntroductionPFAPA syndrome is a benign, non-hereditary condition, of unknown etiology and pathogenesis. There are few reports of it in South America. The purpose of this article is to communicate the experience in a large pediatric hospital in Argentina.Patients and methodsA total of 18 patients were diagnosed with PFAPA between 2002 and 2009 at the Medium Risk Clinic, Prof. Dr. Juan P. Garrahan Pediatrics Hospital, Buenos Aires City. The modified criteria reported by Thomas et al were used for diagnosis. The follow up continued with evaluations during new febrile episodes, clinic check ups and telephone calls.ResultsThe mean age at onset of symptoms was 2.5 years (range: 0.4–7.5) and the mean lag time from onset of symptoms and diagnosis was 3.2 years (range: 0.4-10.9). Fever episodes lasted for a mean of 4.5 days (range: 2-8), with a mean interval of 23 days (range: 15-30) between the beginning of the attacks. Febrile episodes were treated with methyl prednisone at a dose of 1 mg/kg or betamethasone at a dose of 0.15 mg/kg in a single dose. With a mean follow up of 2.6 years (range: 0.5–5.9) 13 patients remain with febrile episodes at a mean interval of 4.6 months (range: 1-12). Five patients did not have febrile crisis for more than a year during the study period and they are considered cured; in this group the disease lasted a mean of 4.7 years (1-9.7).ConclusionsPFAPA syndrome is a sporadic, difficult to diagnose, condition. Both methyl-prednisone and betamethasone have shown to be effective in controlling the symptoms during the febrile crisis. The definitive remission of the syndrome may occur in the first years of onset, although in most patients the febrile episodes continue with an increase of free intervals and attenuated symptoms, and full recovery in prepuberty or adolescence with no sequelae.