Autoinflammatory diseases are characterized by seemingly unprovoked episodes of inflammation, without high-titer autoantibodies or antigen-spcific T cells. The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a non-hereditary autoinflammatory syndrome characterized by periodic episodes of these conditions. It usually manifests itself in early childhood, especially before 5 years of age, and lasts several years. Its etiology is unknown, but in a recent study, the efficacy of tonsillectomy was suggested in the PFAPA syndrome. In one of our cases, a 4-year-old boy diagnosed clinically with the PFAPA syndrome, tonsillectomy proved effective. The tonsil is deeply associated with the etiology of autoinflammatory disorders in PFAPA syndrome. These data suggest the possibility that PFAPA may be a new member of the tonsillar focal diseases.
