Clinical characteristics and quality of life in children with pfapa syndrome and Behçet's disease

Abstract

Objective Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome and Beh & ccedil;et's disease (BD) are non-monogenic autoinflammatory disorders with common clinical characteristics and genetic features. We aimed to review both patients' clinical characteristics and quality of life. Methods Retrospective data were collected on patients' clinical and laboratory characteristics with PFAPA and BD between 2019 and 2022. The quality of life questionnaire (Pediatric Quality of Life Inventory) form was completed by the parents of all patients and the control group. Results The study included 150 cases aged between 2 and 18, including 60 with PFAPA, 30 BD patients and 60 healthy volunteers. The duration of attacks in males with PFAPA was longer than that in females (P = 0.015). During attacks, the mean acute phase reactants of PFAPA patients were higher than those of BD (P = 0.010). In addition, there was a statistically significant relationship between the use of colchicine and a decrease in attack frequency in BD patients at 89.29% (P = 0.001 < 0.05) and in PFAPA patients at 88% (P = 0.001 < 0.05). Precisely, 61.67% of PFAPA (n = 37) and 73.33% of BD patients (n = 22) exhibited poor quality of life regarding daily activities and school attendance. Conclusion Limited data on the quality of life of paediatric BD and PFAPA syndrome are available. During the attacks of patients with PFAPA and BD, acute-phase reactants were higher in PFAPA patients. Colchicine is an effective medication in reducing attacks in both groups. Effective and timely treatment is indispensable to improve quality of life.