Issue 8/2026
Stefanov¹, St., Temelkova², К., Vasilev², Т.
¹ Department of Pediatrics, Heart and Brain Hospital, Pleven
² Clinic of Pediatric Rheumatology, Children’s Hospital „Prof. Ivan Mitev“, Medical University -Sofia
PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis) is the most common periodic fever syndrome in childhood and represents an autoinflammatory disorder with a still incompletely understood pathogenesis. Despite its characteristic clinical presentation, the condition is frequently underrecognized, leading to unnecessary antibiotic treatment and delayed diagnosis. This review summarizes current knowledge on the etiology, pathogenesis, epidemiology, and clinical manifestations of PFAPA syndrome, with emphasis on diagnostic criteria, differential diagnosis, and practical aspects of the diagnostic approach. Current therapeutic strategies are discussed, including the use of corticosteroids for acute attacks, colchicine and cimetidine as preventive treatment, the role of tonsillectomy, and biologic therapy in selected refractory cases. Improved awareness of the disease facilitates early diagnosis, reduces unnecessary investigations and treatments, and contributes to better clinical outcomes and quality of life for affected children and their families.
Key words: PFAPA syndrome; periodic fever; autoinflammatory diseases;
Address for correspondence:
Prof. Dr. Stefan Nedev Stefanov
Clinic of Pediatrics, Heart and Brain Hospital – Pleven
2, „Pierre Curie“ Str.
5800, Pleven
tel. +359 888 503 433
e-mail: snsnedev@abv.bg