PFAPA syndrome is not a sporadic disease.

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Serval ID
serval:BIB_5934FCFBDD4F
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
PFAPA syndrome is not a sporadic disease.
Journal
Rheumatology
Author(s)
Cochard Marie, Clet Johanna, Le Lan, Pillet Pascal, Onrubia Xavier, Guéron Thierry, Faouzi Mohammed, Hofer Michaël
ISSN
1462-0332
Publication state
Published
Issued date
2010
Peer-reviewed
Oui
Volume
49
Number
10
Pages
1984-1987
Language
english
Abstract
OBJECTIVES: To determine whether PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) patients have a positive family history (FH) for recurrent fever syndromes.
METHOD: For all patients with PFAPA seen in two paediatric rheumatology centres (Romandy, Switzerland and Bordeaux, France), parents were interviewed to record the FH for periodic fever. As controls, we interviewed a group of children without history of recurrent fever.
RESULTS: We recruited 84 patients with PFAPA and 47 healthy children. The FH for recurrent fever (without an infectious cause and recurring for at least half a year) was positive in 38/84 (45%), and was positive for PFAPA (diagnosis confirmed by a physician) in 10/84 (12%) of the PFAPA patients. For 29 of the 38 patients with positive FH, the affected person was a sibling or a parent. None of the healthy children had a positive FH for recurrent fever or PFAPA. A positive FH for rheumatological diseases was seen in both groups of children.
CONCLUSION: These data show that a significant percentage of PFAPA patients present a positive FH of recurrent fever and PFAPA. This familial susceptibility suggests a potential genetic origin for this syndrome.
Keywords
Heredity, Child, Auto-inflammatory disease, Periodic fever, periodic fever, aphthous stomatitis, adenitis pfapa, pharyngitis, mefv, siblings
Pubmed
Web of science
Open Access
Yes
Create date
20/10/2010 16:28
Last modification date
25/09/2019 7:09
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