Periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenitis (PFAPA) syndrome is the most frequent cause of periodic fever in childhood. The pathogenesis of PFAPA is still unknown. Differantial diagnosis must be made with cyclic neutropenia and other autoinflammatory diseases. Because PFAPA is self limiting and benign, there is no certain treatment model. Treatment options must be specific to the patient, with a strong family and doctor relationship.
Keywords: Periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenitis syndrome, diagnosis, therapy
Periyodik ates, aftoz stomatit, farenjit ve servikal lenfadenit (PFAPA) sendromu cocukluk caginda gorulen, en sik tekrarlayan ates nedenidir PFAPA patogenezi halen tam olarak bilinmemektedir. Siklik notropeni ve diger monogenik otoenflamatuvar hastaliklarla ayirici tanisinin yapilmasi gerekmektedir Kendini sinirlayan ve benign bir hastalik oldugu icin henuz netlik kazanmis bir tedavi sekli yoktur Tedavi seceneklerinin ise guclu bir ebeveyn-doktor iliskisi yardimiyla hastaya ozel olarak secilmesi gerekmektedir.
Anahtar Kelimeler: Periyodik ates, aftoz stomatit, farenjit ve servikal lenfadenit sendromu, tani, tedavi
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Lymphadenitis Syndrome
Periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenitis (PFAPA) syndrome is the most frequent cause of recurrent fevers in childhood. It was first described by Marshall et al. (1) in 1987. PFAPA is diagnosed with recurrent episodes of fever continuing for 3-6 days and following 3 to 8-week intervals; with at least one of the following findings: Aphthous stomatitis, cervical lymphadenitis, and pharyngitis. The disease often starts before the age of 5 and mostly ends in adolescence. The general condition, growth and development of patients are normal between the episodes (1,2).
Most of the patients are between the ages of 2 and 5 with mild male predominance and no racial or ethnic predominance (3). Rarely, familial cases have been reported (4,5). In the majority of patients the attacks last until the age of 10, but it has also been reported in adults (6)
Autoinflammatory syndromes are a group of diseases characterized by inflammatory attacks that occur independently of autoreactive T lymphocytes or external triggering agents. The common termination of genes associated with these syndromes is interleukin (IL)-1[beta] activation. Although the pathogenesis of PFAPA syndrome is not fully understood, it is evaluated in autoinflammatory syndromes. Studies have shown that IL-1[beta], IL-18, tumor necrosis factor-[alpha], interferon (IFN)-[gamma] from proinflammatory cytokines increase in the early phase of fever and IL-6 increases rapidly in the late phase (7-12). In contrast, levels of T-cell-associated cytokines IL-7, IL-17 and anti-inflammatory cytokines IL-10 and IL-4 decrease during exacerbation (8). Dysregulation of IL-1 production from monocytes has been shown in patients (9). In addition, increased expression of inflammation-associated genes (AIM2, CASP1) and IL-1 were detected during the episode (11). In the fever episodes, dysregulation in natural immunity together with an increase in Th1 response, and reduction in Th2 response lead to changes in cytokine levels (8-10,12). Increased levels of monocytes, and decreased levels of eosinophiles and lymphocytes are observed during exacerbations (9,10).
Genetic background is one of the most controversial features of this syndrome. Although it is generally accepted as...
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