PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Abstract

Cecilia Lazea,1 Laura Damian,2 Romana Vulturar,3 Calin Lazar1 1Department Pediatrics I, University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania; 2Department of Rheumatology, County Emergency Clinical Hospital Cluj-Napoca, Centre for Rare Musculoskeletal Autoimmune and Autoinflammatory Diseases, Cluj-Napoca, Romania; 3Department of Molecular Sciences, University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, RomaniaCorrespondence: Cecilia Lazea, Department Pediatrics I, University of Medicine and Pharmacy “Iuliu Hatieganu”, 68, Motilor street, Cluj-Napoca, 400370, Romania, Email [email protected]; [email protected]: The aim of this study is to describe a group of Romanian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome.Materials: This consisted of 39 children diagnosed with PFAPA syndrome according to Thomas’ criteria (eight patients with an age at diagnosis 1 year).Methods: Retrospective analysis of the patients with PFAPA syndrome was focused on clinical features, laboratory findings and therapeutic methods. Comparison between the two groups divided by age at onset was also investigated.Results: Median age at onset was 1.58 years, and median age at diagnosis was 2.97 years. The mean interval between episodes was 35.5 days and the mean duration per febrile episode was 4.1 days. The median diagnosis delay was 2.42 years. The patients presented pharyngitis (100%), adenitis (94.8%) and aphthous lesions (66.7%). The frequency of febrile attacks was higher in children with an age at diagnosis under 1 year (p = 0.0287). Younger age was associated with the presence of aphthae. The mean value of C-reactive protein (CRP) was 7.9mg/dl and the mean value of leucocytes was 14,839/mm3. In 95% of patients given oral corticosteroids, remission of symptoms was reported within 24 h. In three patients, tonsillectomy was performed with complete remission of the disease.Conclusion: We present a cohort of children with PFAPA syndrome, with clinical and laboratory features similar to those described in the literature. Febrile attacks had a higher incidence in children with younger age at the onset of the disease. The patients had a favorable response to corticosteroids.Keywords: PFAPA syndrome, periodic fever, child

Keywords

• pfapa syndrome
• periodic fever
• child