Colchicine versus cimetidine: the better choice for Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome prophylaxis, and the role of MEFV gene mutations

Background During childhood, the most common periodic fever is periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. The effective treatment and prevention of febrile attacks improve these patients' and their families' quality of life. However, there is...

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Published in:Pediatric Rheumatology 2022-08, Vol.20 (1), p.1-72, Article 72
Main Authors: Raeeskarami, Seyed Reza, Sadeghi, Payman, Vahedi, Mahdieh, Asna Ashari, Kosar, Mousavi T., Mahdieh, Ziaee, Vahid
Format: Article
Language:English
Subjects:
Analysis
Care and treatment
Child
Cimetidine
Clinical trials
Colchicine
Corticosteroids
Disease prevention
Families & family life
Fever
Gene mutations
Genes
Genetic aspects
Genetic research
Hyperthermia
Independent sample
Laboratories
Lymphadenitis
Mutation
Patients
Pediatrics
Performance evaluation
Pharyngitis
Prednisolone
Rheumatology
Risk factors
Sample size
Steroids
Stomatitis
Stomatitis, Aphthous
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Summary:Background During childhood, the most common periodic fever is periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. The effective treatment and prevention of febrile attacks improve these patients' and their families' quality of life. However, there is no single strategy or evidence-based guideline to manage this syndrome, and most of them are based on consensus treatment plans. Methods This randomized controlled trial was carried out on 67 PFAPA patients referred to three tertiary centers of pediatric rheumatology. The patients were divided into two groups, including group 1 (n = 36) receiving prednisolone plus colchicine and group 2 (n = 31) receiving prednisolone plus cimetidine. Demographic characteristics and the number of febrile episodes were compared between the two groups before and after the intervention. Results In both groups, the number of febrile episodes after the treatment decreased (P [less than or equai to] 0.001). Statistical Analysis showed no significant difference between the two groups (P = 0.88). Moreover, 44 patients from both groups were checked for the MEFV gene. There were no statistical differences between MEFV positive and negative subgroups in response to colchicine (P = 1). Conclusion This study showed that both drug regimens are significantly effective in preventing febrile attacks in PFAPA syndrome, and the presence of a MEFV gene mutation might not be the only significant risk factor for a response to colchicine. Trial registration IRCT, IRCT20191222045847N1. Registered 23 October 2019, Keywords: Fever, Pharyngitis, Child, Colchicine, Cimetidine
ISSN:1546-0096
1546-0096
DOI:10.1186/s12969-022-00733-3