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Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome - PFAPA syndrome.

Review article
Authors Per Wekell
Published in Presse medicale (Paris, France : 1983)
Volume 48
Issue 1 Pt 2
Pages e77-e87
ISSN 2213-0276
Publication year 2019
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages e77-e87
Language en
Keywords Adrenal Cortex Hormones, therapeutic use, Anti-Inflammatory Agents, Non-Steroidal, therapeutic use, Combined Modality Therapy, Diagnosis, Differential, Fever, etiology, Genetic Predisposition to Disease, Hereditary Autoinflammatory Diseases, classification, diagnosis, genetics, physiopathology, Humans, Inflammation, Lymphadenitis, genetics, Mevalonate Kinase Deficiency, diagnosis, Pharyngitis, genetics, surgery, Prognosis, Recurrence, Stomatitis, Aphthous, genetics, Syndrome, Tonsillectomy
Subject categories Pediatrics


Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease with a clinical phenotype characterised by recurrent episodes of fever, systemic inflammation and symptoms and signs depicted in disease acronym. Although PFAPA is the most common autoinflammatory disease among children in many parts of the world, the condition is still an enigma, which include the regular episodes, the prompt responses to corticosteroids, the genetic bases for the familial clustering and therapeutic effects of tonsillectomy. This review explores PFAPA syndrome with the aim of describing the current clinical and scientific understanding of the condition.

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