Özet
Trombotik mikroanjiyopati (TMA) bulguları, sistemik vaskülitli hastalarda nadiren gelişebilmektedir. Bu çalışmada, TMA’nın klinik, laboratuvar ve/veya histopatolojik özelliklerinin geliştiği sistemik vaskülit olgularını derlemeyi amaçladık. Sistemik vaskülit tanısı konulmuş ve vaskülit tanısı anında veya sonrasında TMA’nın klinik, laboratuvar ve/veya histopatolojik özellikleri gelişen hastaların belirlenmesini sağlayacak kapsamlı bir sistematik literatür taraması yapılmıştır. Sistemik vaskülite eşlik eden TMA’lı 61 olgu çalışmaya dahil edilmiştir. Toplam 31 olgu ile TMA ile en sık ilişkili olan sistemik vaskülit grubu antinötrofil sitoplazmik antikor ilişkili vaskülitlerdi. Altmış bir hastanın otuzunda biyopsi ile kanıtlanmış renal TMA mevcuttu. Kırk altı olguda sistemik vaskülit aktifti, 11 hastada vaskülit inaktifti (kalan 4 olguda vasküliti aktivitesi değerlendirilememiştir). Bu olguların içinde ilaçlar ve aşılar (9 olgu), trombotik trombositopenik purpura (8 olgu), preeklampsi dahil olmak üzere hipertansiyon (8 olgu), atipik hemolitik üremik sendrom (5 olgu) ve sitomegalovirüs (2 hasta) gibi TMA’nın sekonder nedenleri de mevcuttu. TMA tedavisi için 52 hastaya glukokortikoidler, 35 hastaya plazma değişimi, 26 hastaya siklofosfamid ve 6 hastaya ekulizumab uygulanmıştır. Sistemik vaskülitli hastalarda sekonder nedenlere bağlı TMA da görülebilir. TMA özellikleri yeni başlayan vaskülit bulgularıyla beraber veya vaskülit nüksü sırasında gelişiyorsa, vaskülit aktivitesi TMA için potansiyel bir tetikleyici olabilir. Aktif vaskülitle ilişkili TMA olgularında immünsüpresiflere ek olarak, sıklıkla plazma değişimi yapılır ve bazı durumlarda ekulizumab ile kompleman inhibisyonu da faydalı olabilir. Bu bulgular, sistemik vaskülitle ilişkili TMA olgularında zamanında teşhisin ve bireyselleştirilmiş tedavinin daha iyi sonuçlar almak için ne kadar önemli olduğunu vurgulamaktadır.
Anahtar Kelimeler:
Sistematik literatür taraması, trombotik mikroanjiyopati, vaskülit
Kaynaklar
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