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.
D000328:Adult; D001323:Autoantibodies / Q000302:isolation & purification*; D002648:Child; D002675:Child, Preschool; D003602:Cytotoxicity, Immunologic; D004730:Endothelium, Vascular / Q000276:immunology*; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007074:Immunoglobulin G / Q000302:isolation & purification; D007075:Immunoglobulin M / Q000302:isolation & purification; D007223:Infant; D007371:Interferon-gamma / Q000627:therapeutic use; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000276:immunology* / Q000628:therapy
D001323:Autoantibodies; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007371:Interferon-gamma