Development of Kawasaki disease in a patient with PFAPA

Takahito Ninomiya, Hidetoshi Takada, Yusaku Nagatomo, Etsuro Nanishi, Hazumu Nagata, Kenichiro Yamamura, Takehiko Doi, Kazuyuki Ikeda, Toshiro Hara

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases of unknown etiology characterized by regularly recurrent fever episodes with attacks lasting 3-6 days every 3-8 weeks associated with at least one of the three cardinal clinical signs: aphthous stomatitis, pharyngitis, and cervical adenitis. Kawasaki disease (KD) is an acute, self-limited systemic vasculitis that occurs predominantly in infants and young children. In most KD patients, i.v. immunoglobulin leads to a rapid amelioration of clinical symptoms and significantly decreases the risk of coronary artery aneurysms. Although the etiology of KD is still unknown, it was reported that innate immunity was activated in the patients. Described herein is a patient with PFAPA who developed KD. This is the first report of KD development in a PFAPA patient. The association between KD and PFAPA may represent a genetic predisposition to dysregulated innate immune response.

Original languageEnglish
Pages (from-to)801-802
Number of pages2
JournalPediatrics International
Issue number6
Publication statusPublished - Dec 2013

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health


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