Five cases of PFAPA syndrome treated with tonsillectomy

Abstract

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis （PFAPA） syndrome is an autoinflammatory disease and is generally seen in children under 5 years of age. Because the etiology and pathogenesis of PFAPA syndrome are unknown, the syndrome is often misdiagnosed as recurrent tonsillitis. Medication is normally chosen for the first treatment, but tonsillectomy is considered when the medication is ineffective. Herein, we report five cases of PFAPA syndrome who underwent tonsillectomy in our department. The age of onset ranged from 0 to 5 years （median 3 years）, and the age at tonsillectomy ranged from 2 to 7 years （median 5 years）. Symptoms included periodic fever in 5 cases, aphthous stomatitis in 1 case, cervical lymphadenitis in 2 cases, and pharyngitis in 5 cases. After tonsillectomy, the periodic fever disappeared in four cases, and the fever onset became less frequent in the remaining case. Therefore, tonsillectomy was effective in the treatment of PFAPA syndrome. When patients are introduced by a pediatrician who suggests that tonsillectomy is indicated because of failure of the initial treatment, we should recommend the operation for the patients.