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Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.
Int J Rheum Dis. 2019 Aug; 22(8):1489-1497.IJ

Abstract

AIM

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a common inflammatory disease that presents with periodic fever. We aimed to establish more specific diagnostic criteria for PFAPA based on the clinical characteristics of PFAPA patients in our directory.

METHOD

The clinical, laboratory, genetic, and family history details of 257 Japanese PFAPA patients treated at our and other affiliated hospitals between April 2000 and April 2018 were analyzed along with quantitative measurements of the number of CD64 molecules on neutrophils, and the levels of serum inflammatory cytokines. The sensitivity and specificity of the criteria were calculated for several diseases.

RESULTS

Because recurrent fevers were crucial findings, they were defined as the required criterion. Tonsillitis/pharyngitis with white moss were important accompanying signs. Other symptoms associated with febrile episodes were cervical lymphadenitis with tenderness, aphthous stomatitis, sore throat, vomiting, and headache but not cough. A total of 159 (62%) patients had a family history of recurrent fevers, indicating autosomal dominant inheritance. C-reactive protein levels were extremely elevated during febrile attacks but normal in attack-free periods. Serum immunoglobulin D levels were high in 72 of the 199 tested patients. Oral glucocorticoid and cimetidine were extremely effective in all and 51.6% of the patients, respectively. We defined the above as supportive criteria. These criteria were sensitive and specific enough to distinguish PFAPA from other recurrent fever diseases. Raised serum interferon-γ levels and remarkable CD64 expression on neutrophils during flare-ups were recognized, indicating they contributed to diagnosis.

CONCLUSION

Our new criteria are useful for diagnosing PFAPA.

Authors+Show Affiliations

Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.Department of Pediatrics, Kofu Municipal Hospital, Kofu, Japan.Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.Department of Pediatrics, Azumino Red Cross Hospital, Azumino, Japan.Department of Pediatrics, Azumino Red Cross Hospital, Azumino, Japan.Children's Medical Center, Northern Yokohama Hospital, Showa University, Yokohama, Japan.Department of Infection and Host Defense, Graduate School of Medicine, Shinshu University, Matsumoto, Japan.Department of Pathology, Proteo-Science Center and Graduate School of Medicine, Ehime University, Toon, Japan.Department of Pediatrics, Dokkyo Medical University, Shimotsuga, Japan.Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan.Department of Community Health and Preventive Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.Children's Medical Center, Northern Yokohama Hospital, Showa University, Yokohama, Japan.Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan. Children's Medical Center, Northern Yokohama Hospital, Showa University, Yokohama, Japan. Department of Infection and Host Defense, Graduate School of Medicine, Shinshu University, Matsumoto, Japan.

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

31131563

Citation

Takeuchi, Yusuke, et al. "Clinical Features and New Diagnostic Criteria for the Syndrome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis." International Journal of Rheumatic Diseases, vol. 22, no. 8, 2019, pp. 1489-1497.
Takeuchi Y, Shigemura T, Kobayashi N, et al. Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Int J Rheum Dis. 2019;22(8):1489-1497.
Takeuchi, Y., Shigemura, T., Kobayashi, N., Nagumo, H., Furumoto, M., Ogasawara, K., Fujii, H., Takizawa, M., Soga, T., Matoba, H., Masumoto, J., Fukushima, K., Migita, K., Ojima, T., Umeda, Y., & Agematsu, K. (2019). Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. International Journal of Rheumatic Diseases, 22(8), 1489-1497. https://doi.org/10.1111/1756-185X.13610
Takeuchi Y, et al. Clinical Features and New Diagnostic Criteria for the Syndrome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis. Int J Rheum Dis. 2019;22(8):1489-1497. PubMed PMID: 31131563.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. AU - Takeuchi,Yusuke, AU - Shigemura,Tomonari, AU - Kobayashi,Norimoto, AU - Nagumo,Haruo, AU - Furumoto,Masahiro, AU - Ogasawara,Kyo, AU - Fujii,Hitomi, AU - Takizawa,Masahiro, AU - Soga,Takashi, AU - Matoba,Hisanori, AU - Masumoto,Junya, AU - Fukushima,Keitaro, AU - Migita,Kiyoshi, AU - Ojima,Toshiyuki, AU - Umeda,Yoh, AU - Agematsu,Kazunaga, Y1 - 2019/05/27/ PY - 2018/11/27/received PY - 2019/05/03/revised PY - 2019/05/05/accepted PY - 2019/5/28/pubmed PY - 2020/1/30/medline PY - 2019/5/28/entrez KW - PFAPA KW - cytokine KW - diagnostic criteria KW - periodic fever KW - tonsillitis SP - 1489 EP - 1497 JF - International journal of rheumatic diseases JO - Int J Rheum Dis VL - 22 IS - 8 N2 - AIM: The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a common inflammatory disease that presents with periodic fever. We aimed to establish more specific diagnostic criteria for PFAPA based on the clinical characteristics of PFAPA patients in our directory. METHOD: The clinical, laboratory, genetic, and family history details of 257 Japanese PFAPA patients treated at our and other affiliated hospitals between April 2000 and April 2018 were analyzed along with quantitative measurements of the number of CD64 molecules on neutrophils, and the levels of serum inflammatory cytokines. The sensitivity and specificity of the criteria were calculated for several diseases. RESULTS: Because recurrent fevers were crucial findings, they were defined as the required criterion. Tonsillitis/pharyngitis with white moss were important accompanying signs. Other symptoms associated with febrile episodes were cervical lymphadenitis with tenderness, aphthous stomatitis, sore throat, vomiting, and headache but not cough. A total of 159 (62%) patients had a family history of recurrent fevers, indicating autosomal dominant inheritance. C-reactive protein levels were extremely elevated during febrile attacks but normal in attack-free periods. Serum immunoglobulin D levels were high in 72 of the 199 tested patients. Oral glucocorticoid and cimetidine were extremely effective in all and 51.6% of the patients, respectively. We defined the above as supportive criteria. These criteria were sensitive and specific enough to distinguish PFAPA from other recurrent fever diseases. Raised serum interferon-γ levels and remarkable CD64 expression on neutrophils during flare-ups were recognized, indicating they contributed to diagnosis. CONCLUSION: Our new criteria are useful for diagnosing PFAPA. SN - 1756-185X UR - https://www.unboundmedicine.com/medline/citation/31131563/Clinical_features_and_new_diagnostic_criteria_for_the_syndrome_of_periodic_fever_aphthous_stomatitis_pharyngitis_and_cervical_adenitis_ L2 - https://doi.org/10.1111/1756-185X.13610 DB - PRIME DP - Unbound Medicine ER -