Periodic fever with aphthous stomatitis, pharyngitis and adenitis: report of 21 cases.

Isabel Sampaio, José Gonçalo Marques


PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) is a benign sporadic syndrome of unknown cause and an important diagnosis to consider in the child with recurrent fever and tonsillitis. Objectives: To describe the presentation, age of onset, time until diagnosed, episode duration, frequency of typical and atypical symptoms, clinical course after diagnosis and response to treatment in a case series of children with PFAPA.Case series of children with PFAPA referred to our consult over an eight-year period (from May 2001 to May 2009). Data were collected from medical records and telephone calls.We included 21 patients with PFAPA, with age of onset between 6 months and 5 years (median 24 months). Diagnosis was established 4 months to 3 years after onset of crises (median 24 months). Episodes recurred every 2 to 6 weeks (median 30 days), and consisted in 3 to 10 days (median 4,5 days) of fever (21/21), pharyngitis (21/21), cervical adenitis (19/21), and aphthous stomatitis (16/21). Atypical symptoms were reported sporadically and without clinical severity: abdominal pain (8 patients), nauseas/vomits (3 patients), arthralgia (3 patients), hepatosplenomegaly (1 patient), lactose intolerance (1 patient). Eighteen patients received treatment with one dose of prednisolone with rapid symptomatic relief. In two patients subsequent crises became more frequent for a short period of time and then returned to monthly periodicity. Seven treated patients experienced less frequent episodes and in four of them this occurred after diagnosis but before first dose of prednisolone. In the three untreated patients the crises bécame rare and treatment was not prescribed. Tonsillectomy was performed in two patients and in one the monthly episodes reappeared five months after the procedure.In spite including a small number of patients, our case series is similar to others in the literature regarding most clinical aspects. PFAPA syndrome should be considered even in the absence of all clinical criteria. Correct and timely diagnosis does not require and obviates unnecessary diagnostic tests. The outcome after symptomatic therapy with corticosteroids and family reassurance was generally good and we do not recommend tonsillectomy as a first line treatment in this syndrome.

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