Background/Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. Tonsillectomy leads to cessation of febrile episodes in most children, but some continue to have symptoms after surgery. We evaluated a cohort of children with PFAPA who underwent tonsillectomy.

Methods: We characterized clinical features and response to tonsillectomy in 93 children with PFAPA from two different centers, Vanderbilt University Medical Center and the National Institutes of Health (NIH) in the United States, with subject/parent interviews and medical record review. We used multivariable logistic regression to identify predictors of favorable response to tonsillectomy.

Results: The 93 children enrolled in our study were followed for a median of 47 months after tonsillectomy. Overall, 48% had complete resolution of PFAPA episodes (65% at Vanderbilt, 34% at NIH), 25% had less severe or frequent episodes, 22% had a period of remission with recurrence, and 5% had no change in episodes. Children whose episodes aborted with corticosteroid or who had pharyngitis during flares prior to tonsillectomy were more likely to have a favorable response to surgery. Among those with continued flares after tonsillectomy, 63% (29/46) reported that some or all flares were feverless, and 44% (21/48) reported triggers for flares including physical and mental stress. Sixty-nine percent (33/48) of children with continued flares after tonsillectomy required treatment. Corticosteroid and anakinra were fully or partially effective in aborting flares in 86% and 100% of subjects who received these respective treatments post-tonsillectomy. Flare severity and frequency improved in 15/19 (79%) subjects who took cimetidine and 5/14 (36%) who took colchicine as prophylaxis. Most (67/93, 74%) children continued to have aphthous ulcers after tonsillectomy.

Conclusion: Tonsillectomy reduces episode severity and frequency in children with PFAPA. However, a portion of children with PFAPA, particularly those who do not respond to corticosteroids or who do not have pharyngitis during flares, have incomplete responses to tonsillectomy, potentially representing a subgroup of PFAPA with differences in disease mechanism.  

 This research was supported in part by the Division of Intramural Research of NIAID and NHGRI, NIH and T32 training grants at Vanderbilt University.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcomes-following-tonsillectomy-in-children-with-periodic-fever-aphthous-stomatitis-pharyngitis-and-cervical-adenitis-pfapa-syndrome/