Pediatric Resident Nationwide Children's Hospital Columbus, Ohio, United States
Background: The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common autoinflammatory fever disorder during childhood. Corticosteroids, colchicine, and cimetidine have been used for management with variable results. Tonsillectomy is reserved as an option in patients for refractory PFAPA. Limited data exists evaluating patients’ response to tonsillectomy as treatment for PFAPA.
Objective: Evaluate patients’ response to tonsillectomy as treatment for refractory PFAPA.
Design/Methods: A retrospective chart review was performed at Nationwide Children’s Hospital in Columbus, Ohio, a pediatric tertiary care center to identify patients with PFAPA. ICD-10 codes were used for patients with recurrent fever and PFAPA who were seen by a pediatric infectious disease or pediatric rheumatology specialist between January 1, 2017 and August 10, 2021. Data collected included demographics, clinical and laboratory findings, steroid use, tonsillectomy date, patient follow up, and fever pattern post tonsillectomy.
Results: 704 patients were evaluated for recurrent fever and/or PFAPA. 80 (11%) patients underwent tonsillectomy. All the patients with tonsillectomy had documented periodic fevers, 53 (66%) had documented aphthous stomatitis and pharyngitis. 38 patients were females (1-14 years; average: 6 years) and 42 were males (1-14 years; average 5 years) at the time of initial evaluation. 33(41%) patients received a trial of steroids. 20(25%) patients had no further follow-up. 48 (60%) patients had documented resolution of fever, post tonsillectomy. 73% (8/11) of patients referred by Rheumatology had resolution of fever, 82% (9/11) referred by Infectious disease had resolution of fever and 57% (31/54) referred by ENT had resolution of fever. Of those referred by Sleep Medicine, Neurology, or multiple specialties, 0% (0/4) had resolution of fever. 32 patients (42%) had recurrences of fever episodes and were later diagnosed with either unclassified immunodeficiency, recurrent viral illness, other autoimmune diseases.Conclusion(s): In this retrospective study, documented resolution of fever was 60% post-tonsillectomy even though some patients had only periodic fevers and did not meet classic diagnostic criteria for PFAPA. Of the 66% patients with documented aphthous stomatitis and pharyngitis, 62% had resolution of fever. 25% of these patients were lost to follow up post tonsillectomy thus response to procedure is uncertain in this population.
