Issue 9/2022
M. Ganeva, St. Stefanov
Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome of early childhood. Although it is a self-limited disease, it may negatively affect the quality of life. The diagnosis of PFAPA is mainly based on clinical classification criteria. The most relevant hypothesis for pathogenesis is that dysregulated immune system in a genetically predisposed individual responds to a yet unidentified trigger in an exaggerated way. For management, single-dose corticosteroids during attacks and tonsillectomy remain the most effective therapies, while colchicine is a promising option to decrease attack frequency. Many pediatric patients are often grouped with patients with PFAPA syndrome despite failing to meet diagnostic criteria. Recently, these patients were categorized as syndrome of undifferentiated recurrent fever (SURF).
Address for correspondence:
M. Ganeva
Clinic of Rheumatology, Hematology and Cardiology,
SBALDB, MU – Sofia
11, „Akad. Ivan Evstratiev Geshov“ Blvd.
1606, Sofia, Bulgaria