Síndrome PFAPA (febre periódica, estomatite aftosa, faringite, adenite): análise retrospectiva de 21 casos.

Abstract

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.

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

“…The mean duration of fever episodes (4.1 days) and the mean interval between flares (5 weeks) resembles those described by other studies. 27,33,34,37,39 In our cohort, the younger the age at diagnosis, the shorter the asymptomatic interval between flares (27 days vs 38 days), another finding that has been reported by other studies. 39,40 On the other hand, a comparison between the clinical presentation of paediatric and adult patients with PFAPA has found that the frequency of the flares was significantly higher in paediatric cases, while febrile attack duration was significantly longer in adults.…”

“…Median time between the onset of the disease and diagnosis was 2.4 years, longer than reported by most of other studies, but not unprecedented. 37,38 This long duration between the onset of the diseases and diagnosis should raise awareness among healthcare professionals. The delay in the diagnosis of PFAPA is evidence of the tendency to underdiagnose this disease, and during this period, these children underwent repeated unnecessary antibiotic treatments (which can lead to complications) because the disease is commonly confounded with bacterial tonsillitis.…”