The endothelium participates actively in homeostatic mechanisms such a
s the regulation of vascular tone and maintenance of a non-thrombotic
environment, as well as directing biological responses such as leukocy
te trafficking to inflammatory sites. Disruption of these processes le
ads to disease. In the antiphospholipid antibody syndrome autoantibodi
es provoke the endothelium to develop a prothrombotic surface. In syst
emic vasculitides associated with presence of antineutrophil cytoplasm
antibodies, it is likely that the autoantibodies incite premature neu
trophil activation, disrupted neutrophil-endothelium interactions and
endothelial damage. This review considers how normal endothelial funct
ions may be subverted in disease and how active endothelial responses
may contribute to disease.