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Assessment of Attack Frequency in PFAPA Patients Based on Therapeutic Modalities
Furkan Kalayci1, Metin Yigit1, Aylin Irmak Kuruc1
1Department of Pediatrics, Ankara City Hospital, Ankara, Turkey.
Insights
Tonsillectomy and colchicine significantly reduce periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) attacks, while repeated corticosteroid use increases attack frequency. Tonsillectomy offers superior long-term control compared to colchicine for PFAPA syndrome.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Genetics
Background:
- Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a common autoinflammatory disorder in children.
- Characterized by recurrent episodes of fever, oral ulcers, pharyngitis, and cervical adenitis.
- Current treatment strategies include corticosteroids, colchicine, and tonsillectomy, with varying efficacy.
Purpose of the Study:
- To present the clinical characteristics of patients diagnosed with PFAPA syndrome.
- To compare the effectiveness of corticosteroid therapy, colchicine prophylaxis, and tonsillectomy in managing PFAPA attack frequency.
Main Methods:
- Retrospective analysis of 195 patients diagnosed with PFAPA syndrome between 2017 and 2021.
- Patients were categorized into untreated, corticosteroid, colchicine, and tonsillectomy groups.
- Demographic data, clinical features, laboratory findings, and treatment outcomes were recorded and analyzed.
Main Results:
- No significant difference in pre-treatment attack frequencies was observed among the groups.
- Both tonsillectomy and colchicine significantly reduced PFAPA attack frequency compared to the pre-treatment period (p < 0.001).
- Repeated corticosteroid use (≥3 attacks) was associated with increased attack frequency (p < 0.0001), while tonsillectomy showed a greater reduction in attacks than colchicine (p < 0.001).
Conclusions:
- Corticosteroids are effective for acute attack management but their repeated use may exacerbate PFAPA.
- Colchicine prophylaxis effectively reduces attack frequency in most PFAPA patients.
- Tonsillectomy provides a high rate of disease control for PFAPA syndrome, demonstrating superior efficacy over colchicine in this study.
Aim:
This study aims to present the clinical characteristics of patients with PFAPA syndrome, and to compare the effects of corticosteroid usage, colchicine prophylaxis, and tonsillectomy on the frequency of attacks in patients with PFAPA syndrome.
Methods:
Patients aged between 6 months and 18 years presenting to our Paediatric Rheumatology clinic between 2017 and 2021 who were diagnosed with PFAPA syndrome and followed up for a minimum of 12 months were included in this study. The demographic and clinical characteristics of the patients, laboratory findings, attack durations, and treatments were recorded.
Results:
Our study, comprised of 195 patients, included four groups: untreated (n = 58), corticosteroid (n = 43), colchicine (n = 62), and tonsillectomy (n = 32). There was no significant difference between the treatment groups in terms of attack frequencies before treatment initiation (p > 0.05). When attack frequencies before and after treatment initiation were observed, a decrease in attack frequency compared to the pre-treatment period was observed in the tonsillectomy group (from 12 to 3 attacks) (p < 0.001) and the colchicine group (from 12 attacks to 1 attack) (p < 0.001). It was noted that using steroids during three or more attacks increased attack frequency compared to the pre-treatment period (p < 0.0001). Tonsillectomy resulted in a more significant reduction in attack frequency compared to the colchicine group (p < 0.001).
Conclusions:
Using corticosteroids during an attack has been shown to effectively control attacks. However, the repeated use of corticosteroids increases the frequency of attacks. Colchicine prophylaxis leads to a reduction in attack frequency in the majority of cases. Tonsillectomy can successfully control the disease in most cases.
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