The lowest dose of corticosteroids, which stops the episodes of PFAPA syndrome

Nadim H Nasser, Nadir N Nasir, Mona G Habib, Nadra G Samra

Abstract

Background
The episodes of PFAPA syndrome with their exhausting periodic fever, annoying oral aphthae and sore throat, warrant treatment. Corticosteroids are the most efficacious.

Aims
Providing evidence that a weight-independent, minimal dose of betamethasone, the least ever used, can resolve symptoms of PFAPA syndrome, within few hours.

Methods
In a retrospective case-series analysis study, approved by the relevant ethical committees of Clalit Health Organization, we collected analysed and interpreted data from medical files of children who suffered from PFAPA syndrome, during 1998 until 2015, concerning the lowest effective betamethasone dose, they received and which had abated fever within few hours.

Results
We had diagnosed 132 children of our 2300 children community, as suffering from PFAPA syndrome (rate 6 per cent). Ninety eight per cent of the patients satisfactorily responded, within two-to-twelve hours, to treatment with a minimal one-time weight-independent dose of betamethasone in every flare. The rest of patients (2 per cent) needed an equal second dose to end their first flare during the following twelve hours. Three patients who were four to five-months-old at time of diagnosis, had received 0.1mg dose.

Conclusion
A dose of 0.5mg of betamethasone for children under 5 years of age and a dose of 1.0mg was prescribed for children >5, were effective for prompt resolution of PFAPA flares. We believe that as long as there is an effective lowest dose for treating PFAPA episodes, a great reduction of corticosteroid consumption will be exercisable globally.
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