PFAPA SYNDROME

Authors

  • Yu. M. Andreichyn I. Horbachevsky Ternopil National Medical University, https://orcid.org/0000-0002-7789-4466
  • I. V. Khoruzhyi LC Medical сenter «LORIMED»
  • K. D. Protsyshyn LC Medical сenter «LORIMED»

DOI:

https://doi.org/10.11603/1681-2727.2022.3.13487

Keywords:

PFAPA syndrome, periodic fever, tonsillopharyngitis, aphthous stomatitis, cervical lymphadenopathy, methods of treatment

Abstract

SUMMARY. According to the data of the literature, РFАРА syndrome – periodic fever with tonsillopharyngitis, aphthous stomatitis and cervical lymphadenopathy – was first described by scientists G.S. Marshall et al. in 1987. This syndrome is a common autoinflammatory diseases in children but also occurs in adults in many parts of the world. Its etiology and pathogenesis have not yet been clarified. The search and improvement of diagnostic criteria is ongoing. Today, standardized treatment regimens for patients are needed: 1) nonsteroidal anti-inflammatory drugs, 2) abortive therapy during episodes with glucocorticoids, 3) prophylactic therapy with cimetidine or colchicine, 4) surgical therapy with tonsillectomy. However, relapses of the disease occur.

Author Biographies

Yu. M. Andreichyn, I. Horbachevsky Ternopil National Medical University,

MD, Professor of the Department of Otorhinolaryngology, Ophthalmology and Neurosurgery, I. Horbachevsky Ternopil National Medical University

I. V. Khoruzhyi, LC Medical сenter «LORIMED»

PhD, doctor of otolaryngology at the Medical Center “LORIMED”

K. D. Protsyshyn, LC Medical сenter «LORIMED»

pediatrician at the medical сenter “LORIMED”

References

Marshall, G. S., Edwards, K. M., Butler, J., Lawton A. R. (1987). Syndrome of periodic fever, pharyngitis and aphtous stomatitis. J. Pediatr, 110 (1), 43-46.

Forsvoll, J., Kristoffersen, E. K., Oymar, K. (2013). Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. J. Acta Paediatr, 102, 187-192.

Rydenman, K., Berg, S., Karlsson-Bengtsson, A., Fasth, A., Wekell, P. (2019). PFAPA syndrome – An important differential diagnosis in children with recurrent fever. J. Lakartidningen, 116. https://pubmed.ncbi.nlm.nih.gov/31661147/

Cantarini, L., Vitale, A., Sicignano, L. L., Emmi, G., Verrecchia, E., Patisso, I., ... & Manna, R. (2017). Diagnostic criteria for adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Frontiers in Immunology, 8, 1018.

Takeuchi, Y., Shigemura, T., Kobayashi, N., Nagumo, H., Furumoto, M., Ogasawara, K., ... & 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.

Amarilyo, G., Rothman, D., Manthiram, K., Edwards, K. M., Li, S. C., Marshall, G. S., ... & Lapidus, S. (2020). Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group. Pediatric Rheumatology, 18(1), 1-7.

Levytska, S. A. (2017). PFAPA syndrome in children: remove or keep tonsils? Neonatology, surgery and perinatal medicine, 7, 2(24). http://neonatology.bsmu.edu.ua/article/view/2413-4260.VII.2.24.2017.5

Kubota, K., Ohnishi, H., Teramoto, T., Kawamoto, N., Kasahara, K., Ohara, O., & Kondo, N. (2014). Clinical and genetic characterization of Japanese sporadic cases of periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome from a single medical center in Japan. Journal of clinical immunology, 34(5), 584-593.

Kolly, L., Busso, N., von Scheven-Gete, A., Bagnoud, N., Moix, I., Holzinger, D., ... & Hofer, M. (2013). Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome is linked to dysregulated monocyte IL-1β production. Journal of Allergy and Clinical Immunology, 131(6), 1635-1643.

Stojanov, S., Hoffmann, F., Kéry, A., Renner, E. D., Hartl, D., Lohse, P., ... & Belohradsky, B. H. (2006). Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response. European Cytokine Network, 17 (2), 90-97.

Asna Ashari, K., Rezaei, N. (2021). PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome: an overview of genetic background. J. Clin. Rheumatol., 40 (11), 4437-4444.

Batu E. D. (2019). Periodic fever, aphtous stomatitis, pharyngitits and cervical adenitits (PFAPA) syndrome: main futures and an algorithm for clinical practice. J. Rheumatol. Int., 39 (6), 957-970.

Batu, E. D., Batu, H. B. (2019). Recurrence of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy: case-based review. J. Rheumatol. Int., 39 (6), 1099-1105.

Gozen, E. D., Yildiz, M., Kara, S., Tevetoglu, F., Haslak, F., Adrovic, A., ... & Kasapcopur, O. (2022). Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever. Rheumatology International, 1-9. https://pubmed.ncbi.nlm.nih.gov/36116090/

Published

2023-01-26

How to Cite

Andreichyn, Y. M., Khoruzhyi, I. V., & Protsyshyn, K. D. (2023). PFAPA SYNDROME. Infectious Diseases – Infektsiyni Khvoroby, (3), 63–66. https://doi.org/10.11603/1681-2727.2022.3.13487

Issue

Section

Reviews and lectures