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

• Published in: The Journal of pediatrics 2011-12, Vol.159 (6), p.958-964
• Main Authors: Wurster, Victoria M., BS, Carlucci, James G., MD, Feder, Henry M., MD, Edwards, Kathryn M., MD
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Child, Preschool; Diseases of the osteoarticular system; Facial bones, jaws, teeth, parodontium: diseases, semeiology; Female; Fever; Follow-Up Studies; General aspects; Humans; Infant; Inflammatory joint diseases; Lymphadenitis - diagnosis; Lymphadenitis - therapy; Male; Medical sciences; Neck; Non tumoral diseases; Otorhinolaryngology. Stomatology; Pediatrics; Pharyngitis - diagnosis; Pharyngitis - therapy; Stomatitis, Aphthous - diagnosis; Stomatitis, Aphthous - therapy; Syndrome; Time Factors; Young Adult
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2011.06.004

Objective To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Study design Patients enrolled in a PFAPA registry were contacted and surveyed. Results 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. Conclusions 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.