Periodic fever, aphthous stomatitis, pharyngitis, adenitis: clinical manifestations, response to steroids, and a practice of online diagnosis and follow-up

Background Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, although not rare, is frequently underdiagnosed. This might be related to the fact that some primary care physicians are not familiar with this syndrome. With the application of network technology, internet tools may also be used for remote diagnosis and follow-up of PFAPA. Herein, we present our experience regarding online diagnosis and management, and provide a more detailed clinical profile of PFAPA in children. Methods All children with long-standing recurrent fever, including those seen at our hospital (clinic group) and those managed through the Haodf.com online consultation platform (online group), were enrolled and followed up. Children diagnosed with PFAPA were retrospectively analyzed, with particular attention to response to corticosteroid therapy during episodes. Clinical manifestations were compared between the two groups. Results Among 1,211 patients with PFAPA, febrile episodes lasting 4.0 days (3.0, 4.5) recurred at intervals of 30.0 days (20.0, 30.0), accompanied by exudative tonsillitis (85.5%), leukocytosis (88.6%) and elevated C-reactive protein (95.7%). Prednisone aborted episodes within 3.0 hours (1.0, 4.0); however, 54.2% of patients experienced shortened intervals after prednisone treatment, 38.2% showed no change in intervals, and 7.6% had prolonged cycles. Recrudescence within 24~72 hours was observed in 29.0% of patients who received a single low dose of prednisone. Clinical manifestations were generally similar between the clinic and online groups. At the time of diagnosis, children had experienced recurrent episodes for a median of 12.0 months (6.0, 12.0), yet only 18.4% had previously been suspected of or diagnosed with periodic fever or PFAPA. Conclusions Oral prednisone is highly effective in aborting attacks but may shorten the interval between episodes in most patients. Underdiagnosis remains common. Online diagnosis and follow-up represent feasible and convenient approaches to PFAPA management.