Kompleman Faktör H Antikoru İlişkili Atipik Hemolitik Üremik Sendrom ve Ekulizumab Tedavisi

Abstract

Atipik hemolitik üremik sendrom aHÜS , sıklıkla son dönem böbrek yetersizliğine ilerleyen ender görülen kro- nik bir hastalıktır. Bu hastaların çoğunda alternatif komp- leman yolağının bozulmasına neden olan genetik veya edinsel bozukluk vardır. Kompleman Faktör H otoantikor- ları aHÜS’lü vakaların %6-11’inde bildirilmiştir. Plazma değişimi ve immunsupresif tedaviye rağmen, relapsları önlenemeyen, ancak Ekulizumab kullanımı ile remisyon sağlanan bir anti-FH antikor ilişkili aHÜS vakasını sun- mayı amaçladık

Keywords

• Atipik hemolitik üremik sendrom,kompleman faktör H antikorları,Ekulizumab tedavisi

Complement Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome and Eculizumab Treatment

Abstract

Atypical hemolytic uremic syndrome aHUS is a rare, chronic disease with frequent progression to end-stage renal disease. In the majority of these patients, there is a genetic or acquired disorder that causes the dysregulation of the alternative complement pathway. Factor H autobodi- es have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibody- associated aHUS with intractable relapses despite plasma exchange and immunosuppressive treatment, but remission was provided by the use of eculizumab

Keywords

• Atypical hemolytic uremic syndrome,factor H autoantibodies,eculizumab therapy

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