Seven cases of Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease. It often occurs in children ranging from 3 to 4 years of age. The episode of fever itself does not affect growth and development, and it often demonstrates spontaneous remission. Tonsillectomy and medication are the alternatives to treat the symptoms. Tonsillectomy leads to a high rate of complete remission, while corticosteroids promptly stop disease episodes and a H₂ blocker, cimetidine can postpone febrile episodes. Of the 7 cases we experienced in our department, 5 cases underwent tonsillectomy, and all cases achieved remission. Medication was also efficient, with fever resolution and extension of the interval between fever episodes. PFAPA syndrome may be diagnosed and treated as chronic tonsillitis, since some of the symptoms are common, but this syndrome should be included in the differential diagnosis in order to expand the treatment options and contribute to improving the QOL of patients and their families.