AB1057 DIFFICULTIES IN THE DIAGNOSIS OF THE PFAPA SYNDROME IN THE REAL CLINICAL PEDIATRICIAN PRACTICE
Authors: Anna Spivakovskaya, Yuri Spivakovskiy, Yuri Cnernenkov, Asiya Dushanova
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Symptom complex of PFAPA syndrome is characterized by a rather diverse clinical picture, including recurrent fever, aphthous stomatitis, pharyngitis, adenitis. The annual incidence of PFAPA syndrome does not exceed 0,2‰ [1], but it is believed that this disease is much more common than diagnosed [2]. The absence of specific genetic and biochemical markers of this syndrome determine the complexity of timely diagnosis and treatment of this auto-inflammatory disease. <h3>Objectives</h3> The object is to identify objective reasons that impede timely diagnosis of PFAPA syndrome in the clinical practice of a pediatrician. <h3>Methods</h3> We conducted a follow-up analysis of the medical documentation of patients with verified PFAPA syndrome who were examined and treated at the hospital pediatrics department of the university clinic for the period from 2015 to 2019 year. A survey of pediatricians and pediatric otolaryngologists was carried out on the knowledge of diagnostic criteria, protocol for the management and treatment of patients with PFAPA syndrome. <h3>Results</h3> During this period, in the department of PFAPA syndrome was diagnosed in 7 patients. Among patients with a diagnosis of PFAPA syndrome, there were 4 boys and 3 girls, the average age of children at the time of diagnosis was 3,4 ± 0,7 years. Before the diagnosis was verified, all patients were observed by a pediatrician or otorhinolaryngologist regarding repeated manifestations of tonsillitis and received antibacterial therapy at each episode of exacerbation of the disease. The average period from the first manifestations of the disease to the verification of the diagnosis was 1,4 ± 0,5 years. Diagnostic algorithm for verification of the diagnosis was completed by setting prednisolone test. According to the results of this test, in 100% of cases it was possible to completely arrest the phenomena of the inflammatory process (fever, tonsillitis, pharyngitis, stomatitis) and to normalize biochemical markers of inflammation (CRP) without the use of antibacterial therapy. With further observation, two of 7 patients recorded a decrease in the effectiveness of glucocorticoid therapy, which required a tonsilectomy, and one of them noted the temporary effectiveness of colchicine. According to the results of a questionnaire survey of pediatricians and children's otorhinolaryngologists of the outpatient service, a low level of knowledge was revealed on the issues of the clinic, diagnosis and therapy of auto-inflammatory diseases. Only 40% of respondents were able to specify the diagnostic criteria for setting PFAPA syndrome and determine the further routing of these patients. It should be noted that more than half of the respondents (57%) had a clear idea about the methods of rational therapy of patients with PFAPA syndrome. <h3>Conclusion</h3> Thus, the complexity of the primary diagnosis of PFAPA syndrome is associated both with the clinical features of the disease and the insufficient level of knowledge of primary outpatient specialists on this issue, which is largely due to the low incidence of PFAPA syndrome. This conclusion was the reason for the inclusion of additional topics in the educational course of pediatricians. <h3>References</h3> [1] Forsvoll J., Kristoffersen E.K., Oymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Paediatrica. 2013; 102: 187–192. [2] Schindera F., Niederhoff H., Schindera S. et al. Periodisches Fieber, aphthose Stomatitis, Pharyngitis, zervikale Adenitis. Das PFAPA-Syndrome. Monatssch. Kinderheilkunde.2000; 148 (6):596-599. <h3>Disclosure of Interests</h3> None declared