Complement factor H antibody-associated atypical hemolytic uremic syndrome and eculizumab treatment
Zeynep Yürük Yıldırım, Alev Yılmaz, Sevinç Emre, İlmay Bilge, Bağdagül Aksu, Aydan Şirin
Abstract
Zeynep Yürük Yıldırım, Alev Yılmaz, Sevinç Emre, İlmay Bilge, Bağdagül Aksu, Aydan Şirin
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 autobodies have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibodyassociated aHUS with intractable relapses despite plasma exchange and immunosuppressive treatment, but remission was provided by the use of eculizumab.
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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 autobodies have been reported in 6-11% of the patients with aHUS. We aimed to present a case with anti-FH antibodyassociated aHUS with intractable relapses despite plasma exchange and immunosuppressive treatment, but remission was provided by the use of eculizumab.
Key concepts: Atypical hemolytic uremic syndrome, Eculizumab, Medicine, Complement system, Thrombotic microangiopathy, Factor H, Immunology, Alternative complement pathway