Autoinflammatory Periodic Fever Syndromes

Abstract

Autoinflammatory periodic fever syndromes are a group of diseases mostly presenting in childhood and characterized by recurrent fever and inflammation attacks as a result of an innate immune system activation. Familial Mediterranean fever (FMF) is the most common periodic fever syndrome in Mediterranean countries such as Turkey. Other hereditary autoinflammatory periodic fever syndromes are classified as rare diseases. In this review, the clinical features, recommended classification criteria and treatment options of FMF, tumor necrosis factor (TNF) receptorassociated periodic fever syndrome (TRAPS), mevalonate kinase (MVK) deficiency, cryopyrin-associated periodic fever syndrome (CAPS), and periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome were evaluated.

Keywords

• Autoinflammatory disease
• periodic fever syndrome
• familial Mediterranean fever
• colchicine

Otoinflamatuar Periyodik Ateş Sendromları

Abstract

Otoinflamatuar periyodik ateş sendromları, bulguları çoğunlukla çocukluk çağında başlayan, tekrarlayan ateş ve inflamasyon atakları ile karakterize doğal immün sistem bozukluğu sonucu ortaya çıkan hastalıklar grubudur. Ailevi Akdeniz ateşi (AAA) Türkiye gibi Akdeniz ülkelerinde en sık görülen periyodik ateş sendromudur. Diğer herediter otoinflamatuar periyodik ateş sendromları nadir hastalıklar olarak sınıflandırılmaktadır. Bu derlemede; AAA, tümör nekroz faktör (TNF) reseptörü ilişkili periyodik ateş sendromu (TRAPS), mevalonat kinaz (MVK) eksikliği, kriyopirin ilişkili periyodik ateş sendromu (CAPS) ve periyodik ateş, aftöz stomatit, farenjit ve adenit (PFAPA) sendromunun klinik özellikleri, önerilen sınıflandırma kriterleri ve tedavi seçenekleri değerlendirilmiştir.

Keywords

• Otoinflamatuar hastalık
• periyodik ateş sendromu
• ailevi Akdeniz ateşi
• kolşisin

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