Zespół PFAPA jako choroba autozapalna? kompendium

Abstract

PFAPA syndrome is an autoinflammatory disease that belongs to the group of periodic fever syndromes in children. It is a rare, polygenic disease that has a tendency to self-limit with age and disappear before adulthood. The most important symptoms of PFAPA are: periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy. Insufficient knowledge about the causes of the disease does not yet allow for the use of causal treatment. Pharmacotherapy (ibuprofen, glicocorticosteroids) is used during relapses to reduce symptoms, but it does not prevent systematic relapses. When necessary, tosillectomy allows for complete remission of the disease.