Lytic anti-endothelial cell antibodies in haemolytic-uraemic syndrome.

Sera from 13 of 14 children with acute haemolytic uraemic syndrome (HUS) contained complement-fixing IgG and IgM antibodies that lysed cultured human umbilical vein endothelial cells. In 3 of 3 sera tested, no lysis of dermal fibroblasts was observed. The endothelial cell antigen was lost after treatment of the cells with gamma interferon. In contrast, only 3 of 5 adult patients with acute, non-relapsing, thrombotic thrombocytopenic purpura (TTP) had lytic anti-endothelial antibodies and only 1 of these recognised an antigen lost upon gamma interferon treatment. None of 32 control sera contained lytic anti-endothelial cell antibodies. These data suggest that HUS involves a disorder of immunoregulation and that a unique class of anti-endothelial cell antibodies is produced that may take part in the pathogenesis of vascular injury in HUS.