HALİSE AKÇA, Ferhat ÇATAL, Semra KARA, Aziz POLAT

Periyodik ateş sendromlar

DERLEME Periyodik Ateş Sendromları Otoinflamatuar hastalıklar erken çocukluk döneminde başlayan monogenik inflamatuar durumlardır. Ailesel Akdeniz ateşi (FMF), mevalonat kinaz eksikliği ve tümör nekrozis faktör reseptör ilişkili sendrom gibi monogenik hastalıklarda periyodik ateşe ilaveten raş, serozit (peri-tonit, plevrit), lenfadenopati ve artrit görülür. Sistemik reaktif (AA) amiloidozis ciddi uzun dönem komplikasyo-nudur. Kriyoprin geninde mutasyon saptanan kriyoprino-patilerde ürtiker benzeri raş, artrit, sensorinöral işitme kaybı, santral sinir sistemi ve kemik tutulumu gibi kronik veya rekürren sistemik inflamasyon bulguları görülür. Piyojenik hastalıklarda deri, eklem ve kemiklerde steril piyojen apseler oluşur. Son olarak Blau sendromu gibi granülomatöz hastalıklarda eklem, deri ve üveada nonkazeifiye granülomatöz inflamasyon görülür. Bu yazıda çocuklarda görülen bu hastalıkların klinik özellik-leri ve uygun tedavi yöntemleri anlatılacaktır.

Periodic fever syndromes

Autoinflammatory diseases are a group monogenic inflammatory conditions characterized by an early onset during childhood. Under the term “periodic fevers” are gathered some monogenic diseases characterized by periodic or recurrent episodes of systemic inflammation causing fever often associated with rash, serositis, lymphadenopathy and arthritis. Cryopyrinopathies are responsible for a spectrum of diseases characterized by a chronic or recurrent systemic inflammation. Other disorders are dominated by the presence of sterile pyogen abscesses prevalently affecting the skin, joints, and bones. Finally, some diseases, are characterized by the appearance of typical noncaseating granulomatous inflammation affecting the joints, skin, and uveal tract. In the present review, we will focus on the clinical presentation of these disorders in childhood and report on the available therapeutic strategies.

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