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.
| Original language | English |
|---|---|
| Pages (from-to) | 801-802 |
| Number of pages | 2 |
| Journal | Pediatrics International |
| Volume | 55 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Jan 1 2013 |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
Cite this
Development of Kawasaki disease in a patient with PFAPA. / Ninomiya, Takahito; Takada, Hidetoshi; Nagatomo, Yusaku; Nanishi, Etsuro; Nagata, Hazumu; Yamamura, Kenichiro; Doi, Takehiko; Ikeda, Kazuyuki; Hara, Toshiro.
In: Pediatrics International, Vol. 55, No. 6, 01.01.2013, p. 801-802.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Development of Kawasaki disease in a patient with PFAPA
AU - Ninomiya, Takahito
AU - Takada, Hidetoshi
AU - Nagatomo, Yusaku
AU - Nanishi, Etsuro
AU - Nagata, Hazumu
AU - Yamamura, Kenichiro
AU - Doi, Takehiko
AU - Ikeda, Kazuyuki
AU - Hara, Toshiro
PY - 2013/1/1
Y1 - 2013/1/1
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=84890463528&partnerID=8YFLogxK
U2 - 10.1111/ped.12225
DO - 10.1111/ped.12225
M3 - Article
C2 - 24330294
AN - SCOPUS:84890463528
VL - 55
SP - 801
EP - 802
JO - Pediatrics International
JF - Pediatrics International
SN - 1328-8067
IS - 6
ER -