Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

DOI Web Site Web Site Web Site PubMed 参考文献34件
  • Yusuke Takeuchi
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan
  • Tomonari Shigemura
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan
  • Norimoto Kobayashi
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan
  • Haruo Nagumo
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan
  • Masahiro Furumoto
    Department of Pediatrics Kofu Municipal Hospital Kofu Japan
  • Kyo Ogasawara
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan
  • Hitomi Fujii
    Department of Pediatrics Azumino Red Cross Hospital Azumino Japan
  • Masahiro Takizawa
    Department of Pediatrics Azumino Red Cross Hospital Azumino Japan
  • Takashi Soga
    Children's Medical Center, Northern Yokohama Hospital Showa University Yokohama Japan
  • Hisanori Matoba
    Department of Infection and Host Defense, Graduate School of Medicine Shinshu University Matsumoto Japan
  • Junya Masumoto
    Department of Pathology, Proteo‐Science Center and Graduate School of Medicine Ehime University Toon Japan
  • Keitaro Fukushima
    Department of Pediatrics Dokkyo Medical University Shimotsuga Japan
  • Kiyoshi Migita
    Department of Rheumatology Fukushima Medical University School of Medicine Fukushima Japan
  • Toshiyuki Ojima
    Department of Community Health and Preventive Medicine Hamamatsu University School of Medicine Hamamatsu Japan
  • Yoh Umeda
    Children's Medical Center, Northern Yokohama Hospital Showa University Yokohama Japan
  • Kazunaga Agematsu
    Department of Pediatrics Shinshu University School of Medicine Matsumoto Japan

書誌事項

公開日
2019-05-27
資源種別
journal article
権利情報
  • http://onlinelibrary.wiley.com/termsAndConditions#vor
DOI
  • 10.1111/1756-185x.13610
公開者
Wiley

この論文をさがす

説明

<jats:title>Abstract</jats:title><jats:sec><jats:title>Aim</jats:title><jats:p>The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a common inflammatory disease that presents with periodic fever. We aimed to establish more specific diagnostic criteria for PFAPA based on the clinical characteristics of PFAPA patients in our directory.</jats:p></jats:sec><jats:sec><jats:title>Method</jats:title><jats:p>The clinical, laboratory, genetic, and family history details of 257 Japanese PFAPA patients treated at our and other affiliated hospitals between April 2000 and April 2018 were analyzed along with quantitative measurements of the number of CD64 molecules on neutrophils, and the levels of serum inflammatory cytokines. The sensitivity and specificity of the criteria were calculated for several diseases.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Because recurrent fevers were crucial findings, they were defined as the required criterion. Tonsillitis/pharyngitis with white moss were important accompanying signs. Other symptoms associated with febrile episodes were cervical lymphadenitis with tenderness, aphthous stomatitis, sore throat, vomiting, and headache but not cough. A total of 159 (62%) patients had a family history of recurrent fevers, indicating autosomal dominant inheritance. C‐reactive protein levels were extremely elevated during febrile attacks but normal in attack‐free periods. Serum immunoglobulin D levels were high in 72 of the 199 tested patients. Oral glucocorticoid and cimetidine were extremely effective in all and 51.6% of the patients, respectively. We defined the above as supportive criteria. These criteria were sensitive and specific enough to distinguish PFAPA from other recurrent fever diseases. Raised serum interferon‐γ levels and remarkable CD64 expression on neutrophils during flare‐ups were recognized, indicating they contributed to diagnosis.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Our new criteria are useful for diagnosing PFAPA.</jats:p></jats:sec>

収録刊行物

参考文献 (34)*注記

もっと見る

関連プロジェクト

もっと見る

キーワード

問題の指摘

ページトップへ