Vol.101 No.5 contentsPRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 101 No. 5 May 2008
PFAPA and Autoinflammatory Diseases
Nobuo Kubo and Kazunari Kaneko
(Kansai Medical University)
PFAPA has recently been defined as a non-hereditary autoinflammatory disease characterized by Periodic episodes of high Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis. PFAPA is a benign pediatric syndrome of unknown etiology. It might have been diagnosed as antibiotic-resistant recurrent tonsillitis and indicated for unnecessary tonsillectomy in the Otolaryngology clinic. Autoinflammatory diseases are a new and expanding classification of inflammatory diseases characterized by recurrent episodes of systemic inflammation in the absence of pathogens, autoantibodies or antigen-specific T cells. This disease is induced by abnormality of neutrophil-related inflammatory cytokines that regulates inflammation, innate immunity and apoptosis. Up-to-date information on innate immunity is also concisely reviewed.
Key words :PFAPA, autoinflammatory diseases, innate immunity