Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

Abstract

To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Patients enrolled in a PFAPA registry were contacted and surveyed. Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses. In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.