Risposta terapeutica alla somministrazione di steroidi in una bambina affetta da PFAPA

PFAPA syndrome is a relatively common periodic fever in children. Etiology and pathophysiology are undefined. It typically starts in early childhood (between ages 2 and 5 years). This syndrome is characterized by febrile episodes lasting 3 to 6 days and recurring about every 3-8 weeks, aphthous stomatitis, pharyngitis and lymphadenopathy. Patients are healthy between episodes and growth is normal. The treatment is based on administration of 1-2 mg/kg of Prednisone in a single dose at start of symptoms. In the not responding cases can be useful the tonsillectomy. Anyway in few years PFAPA syndrome spontaneously disappears without sequelae.