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PFAPA syndrome: A practical review

Journal of Otolaryngology-ENT Research
Tiago Caneira, João Subtil , José Saraiva

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Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is rare, benign recurrent/periodic fever syndrome of unknown etiology that normally affects children under the age of 5. The diagnosis is made based on clinical assumption, after excluding other pathology. In this report to provide a practical assessment of PFAPA syndrome regarding epidemiology, pathogenesis, natural history, clinical manifestations, diagnosis criteria, differential diagnosis, and available medical and surgical treatments.


periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis, syndrome, pfapa, autoinflammatory diseases