A case with periodic fever effectively treated by tonsillectomy after diagnosis of periodic fever with aphthous pharyngitis and adenitis (PFAPA) syndrome

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PFAPA is a non-hereditary autoinflammatory syndrome characterized by periodic episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. It usually manifests in early childhood, especially before 5 years of age, and lasts several years. Its etiology is unknown, but some recent reports suggest a dysregulation of the immune response with continuous pro-inflammatory cytokine activation. We describe a case of periodic fever with few symptoms such as aphthous stomatitis. Thus, diagnosis of the disease was difficult. PFAPA was diagnosed from comprehensive examinations including clinical findings, cytokine profiling, and gene analysis. The episodes of periodic fevers persisted for several years, so tonsillectomy was performed and resulted in marked clinical improvement. In cases of periodic fevers with pharyngitis and cervical adenitis, PFAPA needs to be considered in the differential diagnosis. The management of PFAPA syndrome in children was initially entrusted to pediatricians alone, but now otolaryngologists should be trained to recognize PFAPA syndrome and should consider tonsillectomy, depending on patient's medical conditions.
Japan Society for Pediatric Otorhinolaryngologyの論文