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- Title
The first case of adult-onset PFAPA syndrome in Japan.
- Authors
Satoshi Kutsuna; Norio Ohmagari; Ryutaro Tanizaki; Noboru Hagino; Ryuta Nishikomori; Mugen Ujiie; Nozomi Takeshita; Kayoko Hayakawa; Yasuyuki Kato; Shuzo Kanagawa
- Abstract
A 26-year-old woman presented with fever and pharyngitis. She previously experienced four periodic febrile episodes at 30- to 40-day intervals. We suspected periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, and prescribed predisolone, thereby her fever rapidly subsided. Her febrile episodes improved after daily cimetidine treatment. Genetic testing results of genomic DNA for periodic fever syndromes were negative, although she was heterozygous for p.Glu148Gln variation in MEFV, supporting the diagnosis of PFAPA syndrome.
- Subjects
JAPAN; PHARYNGITIS; AUTOINFLAMMATORY diseases; CIMETIDINE; GENETIC testing
- Publication
Modern Rheumatology, 2016, Vol 26, Issue 2, p286
- ISSN
1439-7595
- Publication type
Article
- DOI
10.3109/14397595.2013.857801