Lütfiye Koru, Elif Selcen Yabancı Erten, Feray Kaya, Elif Küçük, Zelal Aydın, Eda Nur Dizman, Hatice Kübra Dursun, Merve Özen Balcı, Ufuk Furkan Özdemir, Kübra Öztürk, Fatih Haşlak
Trends in Pediatrics - 2025;6(4):268-274
Objective: Based on our clinical observations, this study aimed to evaluate the spectrum of triggers for febrile attacks in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) and to investigate whether clinical characteristics differ by trigger presence or type, providing insights relevant for clinicians managing PFAPA. Methods: The study enrolled children diagnosed with PFAPA by a pediatric rheumatologist according to the European Registry of Autoinflammatory Diseases (EUROFEVER), developed by the Paediatric Rheumatology International Trials Organisation (PRINTO), who had been followed at our tertiary care center for at least six months and were not on prophylaxis. Patients were stratified by the presence of attack triggers. Those with triggers were classified into infection/vaccination, physical/emotional stress, or food intake categories. A comparative analysis of demographic, clinical, and laboratory characteristics was performed between groups during attacks. Results: Triggers were identified in 31.4% of patients (n = 53), most commonly infection/vaccination (16.0%), followed by physical/emotional stress (8.9%) and food intake-related factors (7.1%). Median breastfeeding duration was significantly shorter in the trigger group (17 vs. 24 months, p=0.032). No significant differences were found in the cardinal PFAPA features (oral aphthae, cervical lymphadenitis, and tonsillitis) by trigger status or type. However, constipation during attacks was more frequent in the group with physical/emotional stress ( p=0.020). Conclusion: The clinical phenotype of PFAPA appears largely independent of trigger type. However, shorter breastfeeding duration among patients with triggers suggests early-life factors may influence trigger susceptibility. Additionally, stress-related triggers may link to other symptoms, such as constipation. Recognizing these patterns may help tailor supportive strategies for PFAPA management.
