Author(s): Mano,Lia ; Francisco,Telma ; Gaspar,Joana ; Pereira,Gabriela ; Santos,Raquel ; Abranches,Margarida
Date: 2022
Origin: SciELO Portugal
Subject(s): Atypical Hemolytic Uremic Syndrome/genetics; Atypical Hemolytic Uremic Syndrome/drug therapy; Atypical Hemolytic Uremic Syndrome/immunology; Eculizumab; Influenza Vaccines; Influenza B virus/genetics; Vaccination
ABSTRACT Hemolytic uremic syndrome (HUS) triggered by influenza virus (iHUS) is rare. Influenza A infections have been described to trigger atypical HUS (aHUS) in individuals with an underlying genetic complement dysregulation. To date there are only few reports of Influenza B as a trigger of aHUS, all identified cases associated with mutations in the MCP or C3 gene, occasionally combined with other mutations. aHUS patients should be screened for all known disease-associated genes and screening should not be stopped after finding a mutation, to identify other genetic susceptibility factors influencing gene phenotype, particularly in patients with MCP or CFI mutations. Complement blockade using a monoclonal anti-C5 antibody, eculizumab, has greatly improved the outcome in recent years for certain groups of HUS. The decision on whether to treat or not with eculizumab should be made based on clinical and laboratorial evolution as well as molecular studies results. Influenza A and B are preventable through vaccination and strategies should be addressed for patients with complemente gene mutations identified.
