title: Development of Kawasaki disease in a patient with PFAPA.
abstract: 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.
authors: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors: Takahito
authors: T
authors: Ninomiya
authors: Hidetoshi
authors: H
authors: Takada
authors: Yusaku
authors: Y
authors: Nagatomo
authors: Etsuro
authors: E
authors: Nanishi
authors: Hazumu
authors: H
authors: Nagata
authors: Kenichiro
authors: K
authors: Yamamura
authors: Takehiko
authors: T
authors: Doi
authors: Kazuyuki
authors: K
authors: Ikeda
authors: Toshiro
authors: T
authors: Hara
mesh_terms: D002675:Child, Preschool; D005260:Female; D005334:Fever / Q000150:complications*; D006801:Humans; D008199:Lymphadenitis / Q000150:complications*; D009080:Mucocutaneous Lymph Node Syndrome / Q000209:etiology*; D010507:Periodicity; D010612:Pharyngitis / Q000150:complications*; D013281:Stomatitis, Aphthous / Q000150:complications*; D013577:Syndrome
keywords: Kawasaki disease; aphthous stomatitis; innate immunity; periodic fever; pharyngitis and cervical adenitis syndrome