Follow-up of children with PFAPA syndrome into early adulthood

Abstract

This study represents 12-21 years of follow up of 60 patients who were diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) by strict clinical criteria as young children and were entered into the Vanderbilt registry. The findings add important and predominantly reassuring facts about natural history of PFAPA. The average age of subjects at follow-up was 20 years. Although the majority of patients had resolution of febrile episodes (with mean duration of episodes for 6.3 years), 9 patients continued to have periodic fevers. There was no appreciable difference in age of onset, intervals of well-being, or manifestations during febrile episodes in those who did or did not have resolution of the syndrome during childhood. In patients who had persistent PFAPA >12 years after entry into the registry, duration of current febrile episodes (1.8 days) was shorter and wellness interval (159 days) was longer than at the time of registry. Two patients (both with typical PFAPA symptoms and findings at entry into the registry) subsequently had evolution of symptoms and diagnosis of Behcet disease and familial Mediterranean fever. There was no impairment of growth or development in those whose PFAPA resolved or persisted without another diagnosis.There is much more than is noted above to read and learn about this well-documented cohort with this curious disorder in the full report by Wurster et al. While we await more definitive understanding of the underpinnings of PFAPA syndrome, this report is an invaluable aide to clinicians and families when considering management strategies and anticipating a natural history.Article page 958▶ This study represents 12-21 years of follow up of 60 patients who were diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) by strict clinical criteria as young children and were entered into the Vanderbilt registry. The findings add important and predominantly reassuring facts about natural history of PFAPA. The average age of subjects at follow-up was 20 years. Although the majority of patients had resolution of febrile episodes (with mean duration of episodes for 6.3 years), 9 patients continued to have periodic fevers. There was no appreciable difference in age of onset, intervals of well-being, or manifestations during febrile episodes in those who did or did not have resolution of the syndrome during childhood. In patients who had persistent PFAPA >12 years after entry into the registry, duration of current febrile episodes (1.8 days) was shorter and wellness interval (159 days) was longer than at the time of registry. Two patients (both with typical PFAPA symptoms and findings at entry into the registry) subsequently had evolution of symptoms and diagnosis of Behcet disease and familial Mediterranean fever. There was no impairment of growth or development in those whose PFAPA resolved or persisted without another diagnosis. There is much more than is noted above to read and learn about this well-documented cohort with this curious disorder in the full report by Wurster et al. While we await more definitive understanding of the underpinnings of PFAPA syndrome, this report is an invaluable aide to clinicians and families when considering management strategies and anticipating a natural history. Article page 958▶ Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis SyndromeThe Journal of PediatricsVol. 159Issue 6PreviewTo assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Full-Text PDF