A Potential Link Between Outcome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) Syndrome in Children and Breastfeeding: A Retrospective Single-Center Cohort Snapshot

Background/Objectives: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome, often referred to as PFAPA syndrome, may enigmatically recur for an undetermined time in the affected children: a potential reason to explain its recurring pattern for an unpredictable period and its self-limitation is currently unknown. We explored the relationship of PFAPA symptom evolution during a variable follow-up with PFAPA disease pictures and breastfeeding for less than 6 months during infancy. Methods: In the Outpatients Clinic of Pediatric Rheumatology of our Institution we have retrospectively screened 150 Italian children with history of PFAPA syndrome during the period 2014-2024, all without any recognized chronic diseases: 88 males, 62 females, mean age at onset of 2.5±1.7 years, age range of 0.3-9.4 years, and mean age at diagnosis of 4.5±2.0 years. Results and Conclusions: After dividing patients into two groups based on either the disappearance or persistence of PFAPA symptoms during a variable median follow-up we found that positive family history of recurring fevers, cervical lymphadenopathy, arthralgia, myalgia, and breastfeeding for less than 6 months were associated with the persistence of the disease over time. Inherited autoinflammatory disorders were excluded on a genetic basis in 16.3% of the cohort. Performing a multivariate analysis adjusted for sex and age we found that only breastfeeding for more than 6 months and a higher level of education in PFAPA patients’ mothers were independently associated with the resolution of the recurring PFAPA symptoms.