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[AN ADULT CASE OF PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS (PFAPA) SYNDROME DEVELOPED IN CHILDHOOD AND DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS].
Arerugi. 2020; 69(1):53-58.A

Abstract

We report an adult case of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, who had a tonsillectomy at 10 years old and relapsed later. An early 40's-year-old man had been suffering from recurrent fever attack once in 1-2 months during childhood. He was accompanied by fever which was persist for several days, aphthous stomatitis, tongued tonsillitis with moss, pharyngitis, and submandibular lymphadenitis with tenderness. He was not doing well during fare-up. At the time of admission, CRP level was 12.5mg/dl and the remarkably increased expression of CD64 on neutrophils was found. Bacterial infections and collagen diseases were excluded by the several examinations. We suspected PFAPA syndrome, and treated with cimetidine, but cimetidine was not effective. At the time of flare up, administration of prednisolone was remarkably effective. We diagnosed PFAPA syndrome on the basis of clinical courses. Genetic analysis of responsible gene of familial Mediterranean fever, MEFV showed E148Q heterozygous mutation in exon 2.Since an adult case of PFAPA syndrome is likely to be made misunderstanding for infectious recurrent pharyngitis, it is important to note that we should consider PFAPA syndrome as a differential diagnosis when we meet with the adult patient of recurrent fever.

Authors+Show Affiliations

General Internal Medicine and Infection Control, Iida Municipal Hospital.Department of Molecular and Cellular Immunology, School of Medicine, Shinshu University.Department of Otolaryngology, Iida Municipal Hospital.Department of Otolaryngology, Iida Municipal Hospital.Department of Internal Medicine, Hotaka Hospital.Department of Molecular and Cellular Immunology, School of Medicine, Shinshu University.

Pub Type(s)

Case Reports
Journal Article

Language

jpn

PubMed ID

32051370

Citation

Tsukadaira, Akihiro, et al. "[AN ADULT CASE of PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, and ADENITIS (PFAPA) SYNDROME DEVELOPED in CHILDHOOD and DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS]." Arerugi = [Allergy], vol. 69, no. 1, 2020, pp. 53-58.
Tsukadaira A, Takeuchi Y, Watanabe K, et al. [AN ADULT CASE OF PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS (PFAPA) SYNDROME DEVELOPED IN CHILDHOOD AND DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS]. Arerugi. 2020;69(1):53-58.
Tsukadaira, A., Takeuchi, Y., Watanabe, K., Tsukamoto, K., Okubo, Y., & Agematsu, K. (2020). [AN ADULT CASE OF PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS (PFAPA) SYNDROME DEVELOPED IN CHILDHOOD AND DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS]. Arerugi = [Allergy], 69(1), 53-58. https://doi.org/10.15036/arerugi.69.53
Tsukadaira A, et al. [AN ADULT CASE of PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, and ADENITIS (PFAPA) SYNDROME DEVELOPED in CHILDHOOD and DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS]. Arerugi. 2020;69(1):53-58. PubMed PMID: 32051370.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [AN ADULT CASE OF PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS (PFAPA) SYNDROME DEVELOPED IN CHILDHOOD AND DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS]. AU - Tsukadaira,Akihiro, AU - Takeuchi,Yusuke, AU - Watanabe,Kizuki, AU - Tsukamoto,Koji, AU - Okubo,Yoshio, AU - Agematsu,Kazunaga, PY - 2020/2/14/entrez PY - 2020/2/14/pubmed PY - 2020/3/3/medline KW - CD64 KW - PFAPA syndrome KW - autoinflammatory disease SP - 53 EP - 58 JF - Arerugi = [Allergy] JO - Arerugi VL - 69 IS - 1 N2 - We report an adult case of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, who had a tonsillectomy at 10 years old and relapsed later. An early 40's-year-old man had been suffering from recurrent fever attack once in 1-2 months during childhood. He was accompanied by fever which was persist for several days, aphthous stomatitis, tongued tonsillitis with moss, pharyngitis, and submandibular lymphadenitis with tenderness. He was not doing well during fare-up. At the time of admission, CRP level was 12.5mg/dl and the remarkably increased expression of CD64 on neutrophils was found. Bacterial infections and collagen diseases were excluded by the several examinations. We suspected PFAPA syndrome, and treated with cimetidine, but cimetidine was not effective. At the time of flare up, administration of prednisolone was remarkably effective. We diagnosed PFAPA syndrome on the basis of clinical courses. Genetic analysis of responsible gene of familial Mediterranean fever, MEFV showed E148Q heterozygous mutation in exon 2.Since an adult case of PFAPA syndrome is likely to be made misunderstanding for infectious recurrent pharyngitis, it is important to note that we should consider PFAPA syndrome as a differential diagnosis when we meet with the adult patient of recurrent fever. SN - 0021-4884 UR - https://www.unboundmedicine.com/medline/citation/32051370/[AN_ADULT_CASE_OF_PERIODIC_FEVER,_APHTHOUS_STOMATITIS,_PHARYNGITIS,_AND_ADENITIS_(PFAPA)_SYNDROME_DEVELOPED_IN_CHILDHOOD_AND_DIAGNOSED_AFTER_PROGRESS_MORE_THAN_30_YEARS] L2 - https://dx.doi.org/10.15036/arerugi.69.53 DB - PRIME DP - Unbound Medicine ER -