Adult-onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome Responsive to Colchicine
- PMID: 37062730
- PMCID: PMC10749813
- DOI: 10.2169/internalmedicine.1364-22
Adult-onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome Responsive to Colchicine
Abstract
We herein report a rare case of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome that occurred in an 18-year-old man. He visited our hospital with recurrent episodes of a fever, pharyngitis and adenitis without suggestive findings of infection. These episodes resolved within 5 days and recurred quite regularly, with an interval of about 30 days. As the febrile episodes significantly impaired his quality of life, he was treated with colchicine (0.5 mg) as prophylaxis. This completely prevented the episodes during six months of follow-up. Colchicine may therefore be effective in cases of adult-onset PFAPA syndrome.
Keywords: PFAPA syndrome; adult-onset; colchicine.
Conflict of interest statement
Similar articles
-
Colchicine versus cimetidine: the better choice for Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome prophylaxis, and the role of MEFV gene mutations.Pediatr Rheumatol Online J. 2022 Aug 31;20(1):72. doi: 10.1186/s12969-022-00733-3. Pediatr Rheumatol Online J. 2022. PMID: 36045426 Free PMC article. Clinical Trial.
-
Gray zone in the spectrum of autoinflammatory diseases: familial Mediterranean fever accompanying periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: single-center experience.Eur J Pediatr. 2023 Dec;182(12):5473-5482. doi: 10.1007/s00431-023-05209-4. Epub 2023 Sep 30. Eur J Pediatr. 2023. PMID: 37777601
-
Is colchicine more effective to prevent periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis episodes in Mediterranean fever gene variants?Pediatr Int. 2017 Jun;59(6):655-660. doi: 10.1111/ped.13265. Epub 2017 Apr 24. Pediatr Int. 2017. PMID: 28207965
-
Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India.Front Immunol. 2022 Sep 20;13:958473. doi: 10.3389/fimmu.2022.958473. eCollection 2022. Front Immunol. 2022. PMID: 36203600 Free PMC article. Review.
-
Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.Acta Paediatr. 2019 Aug;108(8):1385-1392. doi: 10.1111/apa.14792. Epub 2019 Apr 14. Acta Paediatr. 2019. PMID: 30901126 Review.
Cited by
-
Adult-Onset PFAPA Syndrome: Successful Management with Colchicine.Am J Case Rep. 2024 Aug 29;25:e943658. doi: 10.12659/AJCR.943658. Am J Case Rep. 2024. PMID: 39205369 Free PMC article.
References
-
- Gaggiano C, Rigante D, Sota J, Grosso S, Cantarini L. Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clin Rheumatol 38: 11-17, 2019. - PubMed
-
- Gattorno M, Hofer M, Federici S, et al. . Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis 78: 1025-1032, 2019. - PubMed
-
- Kolly L, Busso N, von Scheven-Gete A, et al. . Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome is linked to dysregulated monocyte IL-1β production. J Allergy Clin Immunol 131: 1635-1643, 2013. - PubMed
-
- Lachmann HJ. Periodic fever syndromes. Best Pract Res Clin Rheumatol 31: 596-609, 2017. - PubMed
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Medical
Research Materials