Open AccessNew perspectives on the approach to patients with atypical Hemolytic Uremic Syndrome candidates for renal transplantation
Author(s) -
Ana Figueiredo,
Nuno Oliveira,
Catarina Romãozinho,
Helena Sá,
Arnaldo Figueiredo,
Rui Alves
Publication year - 2021
Publication title -
revista portuguesa de nefrologia e hipertensão
Language(s) - English
Resource type - Journals
eISSN - 2183-1289
pISSN - 0872-0169
DOI - 10.32932/pjnh.2021.10.145
Subject(s) - medicine , atypical hemolytic uremic syndrome , eculizumab , nephrology , transplantation , intensive care medicine , blockade , kidney transplantation , disease , end stage renal disease , complement system , immunology , antibody , receptor
Atypical hemolytic uremic syndrome (aHUS) is one of the most challenging diseases for a nephrologist, with high rates of progression to end- -stage kidney disease (ESKD) and post-transplant recurrence. Complement dysregulation has been found in up to 70% of cases, which can be hereditary or acquired. Over the last few years, knowledge of the pathogenesis of aHUS has greatly increased, with the unravelling of the complement’s role, providing not only the chance for individualized post-transplant recurrence risk assessment, but also the possibility of a highly effective treatment through pharmacological C5-9 blockade with eculizumab. The overall outcome and prognosis of patients with aHUS has dramatically improved since the approval of this drug in 2011, allowing renal transplant to be a much safer option for these patients. Our aim was to present a proposal for the management of patients with aHUS, candidates for renal transplantation, in the light of the most recent studies.