AB0052 HEALTH-RELATED QUALITY OF LIFE IN CHILDREN WITH PFAPA SYNDROME AND BEHCET’S DISEASE
Authors: A. Gürel Bedir, S. S. Kilic
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> PFAPA syndrome is a non-monogenic autoinflammatory disorder characterized by periodic fever, aphthous stomatitis, pharyngitis, and adenitis. Some common variants are associated with two oropharyngeal ulcerative disorders, Behcet's disease, and PFAPA syndrome. <h3>Objectives:</h3> Because of shared certain clinical characteristics and genetic features among patients with PFAPA and JBD, we aimed to review both patients' clinical characteristics and quality of life. <h3>Methods:</h3> Retrospective data collection was performed on the clinical and laboratory characteristics of patients with PFAPA and Behcet's disease who were followed up at a tertiary pediatric rheumatology clinic between January 2019 and December 2022. The PedsQL questionnaire form was completed by the parents of all patients diagnosed with PFAPA or juvenile Behcet's disease and the control group. <h3>Results:</h3> The study included 150 cases aged between 2 and 18 years, including 60 previously diagnosed PFAPA patients, 30 juvenile Behcet's patients, and 60 healthy volunteers. The mean age at diagnosis was 29 months in the PFAPA group and 114 months in the juvenile Behcet's group. The duration of attacks in males with PFAPA was longer than that in females (p=0.015). In addition, the use of colchicine was found to be effective in reducing attack frequency by 74.2% for PFAPA patients and 59.8% for Behcet patients (p=0.001&lt;0.05). During attacks, the mean ANS, CRP, and serum amyloid A levels of PFAPA patients were higher than those of Behcet patients (p=0.010). ROC analysis was used to obtain a prediction value for the poor quality of life for PFAPA and juvenile Behcet's patients. Based on the ROC analysis results, the prediction value was determined to be a total scale score of &lt;74.60 for PFAPA patients and a scale total score of &lt;72.50 for juvenile Behcet's patients. Accordingly, 61.67% of PFAPA patients (n=37) and 73.33% of juvenile Behcet's patients (n=22) exhibited poor quality of life. <h3>Conclusion:</h3> There is limited data available on the quality of life of pediatric Behcet's disease and PFAPA syndrome. PFAPA and Juvenile Behcet's disease are both characterized by recurrent attacks, and they may have an impact on the quality of life due to factors such as early age of onset in PFAPA, the need for lifelong treatment in Behcet's disease, the requirement for regular medical check-ups, and the need to avoid school and play environments. <h3>REFERENCES:</h3> [1] Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110: 43- 6. [2] Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J. 2016 Jun 27;14(1):38. [3] Gunes M, Cekic S, Kilic SS. Is colchicine more effective to prevent periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis episodes in Mediterranean fever gene variants? Pediatr Int. 2017;59(6):655-660. [4] Varni JW, Burwinkle TM, Seid M, Skarr D. The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr. 2003; 3(6):329-41. [5] Varni JW, Seid M, Rode CA. The PedsQL measurement model for the Pediatric Quality of Life Inventory. Med Care, 1999,37 126-139. [6] Zhou ZY, Chen SL, Shen N, Lu Y. Cytokines and Behcet's disease. Autoimmun Rev 2012;11(10):699-704. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>