Five cases with PFAPA syndrome

DOI 16 References
  • Inoue Maki
    Department of Otorhinolaryngology, Kanagawa Children's Medical Center
  • Ogahara Noboru
    Department of Otorhinolaryngology, Kanagawa Children's Medical Center
  • Tanabe Teruhiko
    Department of Otorhinolaryngology, Kanagawa Children's Medical Center

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  • 当科における PFAPA 症候群の検討

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  PFAPA syndrome is a non-hereditary auto-inflammatory syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. It manifests in early childhood, generally prior to the age of 5 years. The etiology of PFAPA syndrome is unknown. Antibiotics are not effective, and the therapeutic strategies have not been established yet. However, corticosteroid, cimetidine, and tonsillectomy are suggested to be effective. We treated five cases with PFAPA syndrome at the Department of Otorhinolaryngology of Kanagawa Children's Medical Center from 2008 to 2011. Only one case was treated with corticosteroid and cimetidine. Nevertheless, the symptoms of all five cases persisted, and all cases underwent tonsillectomy. After tonsillectomy, their symptoms were reduced remarkably. We recommend that tonsillectomy should be considered when conservative treatment is not effective.

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