Understanding PFAPA Syndrome in Palestine: A Retrospective Cohort Analysis of Epidemiological and Clinical Data.

Abstract

Background: Despite Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy being widely acknowledged as a clinical entity and considered one of the most prevalent autoinflammatory diseases, there remains controversy surrounding its diagnostic criteria. Additionally, the epidemiology of the disease is largely unknown in Palestine. Therefore, the goal of this study is to enhance the understanding of PFAPA syndrome in Palestinian paediatric patients.

Methods: We carried out a retrospective cohort study that included 57 patients diagnosed with PFAPA at hospitals in Hebron, Palestine, specifically at Al-Ahli and the Palestinian Red Crescent Society (PRCS).

Results: The study revealed that PFAPA patients predominantly experienced fever (93.0%) and pharyngotonsillitis (100.0%), with significant associations noted between PFAPA and the presence of pharyngotonsillitis (p=0.006), adenitis (p=0.001), and periodicity. However, no significant associations were found between PFAPA and aphthous stomatitis, abdominal pain, or arthralgia. Patients with PFAPA were significantly less likely to experience diarrhoea (p=0.007) and chest pain (p=0.003). Treatment modalities included steroids (45.6%), tonsillectomy (57.9%), colchicine (91.2%), and antibiotics (56.1%).

Conclusions: This study offers important perception into the clinical characteristics, treatment outcomes, and epidemiology of PFAPA syndrome in Palestinian patients. The findings highlight fever and pharyngotonsillitis as predominant symptoms, along with significant associations observed with adenitis. Treatment approaches involving steroids, tonsillectomy, colchicine, and antibiotics were frequently utilised, with notable responses reported.