Research Information System
32nd European Paediatric Rheumatology Congress, Helsinki, Finland, 17 - 20 September 2025, pp.62-63, (Summary Text)
Introduction: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is the most common periodic fever disorder in children. Tonsillectomy is considered an important option due to its positive effects on the resolution of attacks. Objectives: This study aims to identify clinical and laboratory predictors for recommending tonsillectomy in PFAPA patients. Methods: This retrospective, multicenter, cross-sectional study included 20 pediatric rheumatology centers in Turkey. Patients diagnosed with PFAPA according to the Eurofever/PRINTO classification criteria between January 2019 and January 2024 were included. Demographic, clinical, and laboratory data were recorded. Surgical outcomes and satisfaction were evaluated in operated patients. Results: This study included 1205 PFAPA patients. Tonsillectomy was recommended for 332 patients, and 167 underwent the procedure. After tonsillectomy, 69.5% of patients experienced complete resolution of symptoms, while 26% had partial recovery. Overall, 89.2% of patients were satisfied with the procedure. The absence of exudative tonsillitis during attacks, along with the presence of pharyngitis or abdominal pain, were significant factors influencing a decision to recommend tonsillectomy. Multivariate analysis showed exudative tonsillitis was a negative predictor (p=0.012), while pharyngitis (p<0.001) and abdominal pain (p<0.001) were positive predictors. Longer attack duration was also associated with tonsillectomy (p=0.043). Conclusion: Identifying which PFAPA patients would benefit from tonsillectomy may contribute to more effective disease management. In particular, patients with prolonged attack duration, absence of exudative tonsillitis, and the presence of pharyngitis or abdominal pain may be appropriate candidates for surgical intervention. Given its significant impact on quality of life, tonsillectomy is associated with high patient satisfaction.