PFAPA syndrome: Study of 10 cases

Background and objectives: "PFAPA syndromes" is an autoinflammatory entity consisting of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Its etiology is unknown although a dysregulation in the control of the autoinflammatory response seems to play a role. Although a genetic origin is suspected, no specific mutation has been determined yet. Corticosteroids are the mainstay of the treatment during the acute attacks. However, in long-term follow-up the role of tonsillectomy is controversial. Patients and methods: A retrospective study of the pediatric cases diagnosed with the PFAPA syndrome was performed in our center during the last 4 years. Results: Ten patients were diagnosed with the syndrome who received corticosteroids as the only treatment with improvement and favourable prognosis. Conclusion: PFAPA syndrome is the most common periodic fever disorder described in childhood whose genetic background has not been yet clarified. Our contribution with 10 patients further supports the common existence of this entity and the need to keep it in mind when having recurrent fevers. (C) 2011 Elsevier Espana, S.L. All rights reserved.