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The first case of adult-onset PFAPA syndrome in Japan.
- Source :
-
Modern rheumatology [Mod Rheumatol] 2016; Vol. 26 (2), pp. 286-7. Date of Electronic Publication: 2013 Dec 02. - Publication Year :
- 2016
-
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 :
- Adult
Cimetidine therapeutic use
Cytoskeletal Proteins genetics
Female
Fever drug therapy
Fever genetics
Glucocorticoids therapeutic use
Heterozygote
Humans
Japan
Lymphadenitis drug therapy
Lymphadenitis genetics
Pharyngitis drug therapy
Pharyngitis genetics
Prednisolone therapeutic use
Pyrin
Stomatitis, Aphthous drug therapy
Stomatitis, Aphthous genetics
Syndrome
Treatment Outcome
Fever diagnosis
Lymphadenitis diagnosis
Pharyngitis diagnosis
Stomatitis, Aphthous diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1439-7609
- Volume :
- 26
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Modern rheumatology
- Publication Type :
- Academic Journal
- Accession number :
- 24289199
- Full Text :
- https://doi.org/10.3109/14397595.2013.857801