Assessment of Attack Frequency in PFAPA Patients Based on Therapeutic Modalities.

Abstract

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.