Nörodejenerasyonda Yeni Bir Oyuncu: Sirküler Rna'ların Alzheimer Patogenezindeki İşlevi

Özet

Eozinofilik Granülomatozis ve Polianjitis (EGPA) veya daha çok bilinen adı ile Churg-Strauss sendromu (CSS) etiyolojisi tam olarak tespit edilemeyen, ANCA (antinötrofilik stoplazmik antikor) pozitif vaskülitik hastalıklar içine dahil edilir. Bağışıklık sisteminin nasıl aktifleştiği ve eozinofil ağırlıklı inflamasyonun patogenezi net değildir. İlk olarak erişkin vakalarda otopsi bulguları ile tanı konulmuş olsa da, hastalarda tipik olarak periferik kanda eoizonofili varlığı ve akciğer ile deri tutulumu başta olmak üzere multiorgan tutulumun olması klinik şüphe için yeterlidir. Hastalıkla ilgili daha çok yayının yapılması ve klinik tablo ile ilgili farkındalığın artması ile tanı sayısında artış görülmüştür. Üç faz içinde incelen EGPA olgularında; alerjik faz, eozinofilik faz ve vaskülitik faz erişkin hastalarda tipiktir. ANCA pozitifliği olguların ortalama %60’nda vardı. Çocuk yaş grubunda ANCA pozitifliği ortalaması %30’lardadır. Çocuk hastalarda patogenez farklılığının nedeni, hücresel yanıttan çok humoral yanıtın ağırlıklı olması gibi bir durumla ilişkili olabilir. Özellikle pediatrik populasyonda IgE seviyesi daha yüksek olduğu bilinir. Literatürde pediatrik EGPA olgularında derin ven trombozu, optik nöropati, retinal arter tıkanıklığı ve ülseratif kolon benzeri ülseratif lezyonlarda bildirilmiştir. Pediatrik olgularda esas tutulum (%85-90) akciğer ve sinüs tutulumudur. EGPA erişkin hastalarında steroid kullanımına bağlı iyi yanıtlar alınmakla birlikte, azatioprin ve metotreksat kullanımı ile ilgili yayınlarda bulunmaktadır. Pediatrik olguların az sunumu ile beraber tedavi için yeterince alternatif olmaması yanında, intravenöz immunoglobin ve intravenöz metil prednisolon kullanımın önemli fayda sağladığı bilinmektedir.

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