Long-term management of patients with PFAPA syndrome

To evaluate the long-term treatment results of patients with PFAPA syndrome and to determine their need for tonsillectomy.

The clinical characteristics, treatments and long-term results of 16 patients admitted to the Pediatric and Otorhinolaryngology Clinic between 2015 and 2019 were retrospectively analyzed.

Twelve male and four female patients were examined between 1.5 and 8 years (mean age 4.8 ± 1.1) (75% male, 25% female). The mean duration of attacks was 4.4 ± 1.4 weeks. Twelve patients recovered completely with single-dose steroid therapy. In the 4-year follow-up of 12 patients who were given a single dose of steroid therapy, there were no relapses. Surgical decisions were made for four patients whose attacks did not pass with steroid treatment. Two patients (75%) underwent adenotonsillectomy, and one patient underwent tonsillectomy While three of these patients did not have an attack again, one patient continued to have an attack every 8 weeks. At the age of 9, his attacks were completely resolved spontaneously. All patients had fever. The most common concomitant symptoms were pharyngitis (94%), cervical adenitis (82%) and aphthous stomatitis (77%). Exudative tonsillitis was present in 25% of the patients.

Medical treatment should be the first treatment option in patients with PFAPA syndrome. Although single-dose steroid treatment is effective in these patients, tonsillectomy is an important treatment option in refractory cases.