PFAPAと自己炎症疾患 [in Japanese] PFAPA and Autoinflammatory Diseases [in Japanese]
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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.
- Pract.Otol. (Kyoto)
Pract.Otol. (Kyoto) 101(5), 395-407, 2008-05-01
The Society of Practical Otolaryngology