Long-Term Symptoms in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome after...

To investigate the initial and long-term clinical course of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy regarding fever episodes and nonfebrile PFAPA-related symptoms.

An observational cohort study with retrospective and cross-sectional data based on 86 of 101 patients who underwent tonsillectomy for PFAPA between January 2006 and March 2020 from a cohort of 336 patients diagnosed with PFAPA as children. Data were collected by structured telephone interviews and review of medical records. Parents were interviewed regarding initial response to tonsillectomy and the clinical course following tonsillectomy. Patients, if ≥18 years old, or parents, if patients were younger, were interviewed regarding symptoms present at the time of this long-term follow-up study.

Six months after tonsillectomy, 45 of 86 participants (54%) had no symptoms of PFAPA, 19 (22%) had only nonfebrile PFAPA-related symptoms, 17 (20%) had ongoing but fewer or milder fever episodes, 1 (1%) had ongoing febrile episodes without improvement, and 4 (5%) had missing data because parents were unavailable. In 10 of 45 patients with initial remission, fever episodes reappeared 0.5-4.5 years after tonsillectomy. At long-term follow-up (median 8.8 years [range 2.8-16.1 years] after tonsillectomy), 54 of 86 (63%) had no symptoms of PFAPA, 15 (17%) had febrile episodes, generally with longer intervals between flares than before tonsillectomy, and 17 (20%) had nonfebrile PFAPA-related symptoms.

While PFAPA symptoms resolve or are milder post-tonsillectomy among most of the patients, the long-term outcomes showing residual symptoms among a substantial minority should be considered when evaluating tonsillectomy as a treatment option for PFAPA. Increased awareness of persistent symptoms after tonsillectomy may improve understanding and health care for these patients.