Modulation of Endothelial Cell Function by Normal Polyspecific Human Immunoglobulins (IVIg)

Endothelium is not merely a barrier between the bloodstream and tissue, but, by virtue of their location, endothelial cells (EC) are continuously facing humoral factors and, in some circumstances, actively participate in inflammatory and immunomodulatory responses. The endothelium plays a central role in the immunopathology of several vascular disorders in many inflammatory conditions such as Kawasaki disease, Wegener's granulomatosis, or vasculitides. It may act either as a target for injury or by encouraging the development of lesions because of its anatomical position and physiological function. Bound anti-endothelial cell antibodies (AECA) in kawasaki disease have the potential to mediate endothelial cell injury and lysis via either complement or by more subtle changes in endothelial cell functions. During inflammation, activated EC rapidly synthesize and release chemokines and cytokines, and express, in a few minutes or in a few hours, new adhesion molecules involved in the adhesion, rolling and diapedesis of leukocytes. Patients suffering from systemic inflammatory conditions such as dermatomyositis, and particularly, Kawasaki syndrome, are greatly benefitted from normal polyspecific human immunoglobulin (IVIg) treatment. IVIg has also been used in the treatment of anti-neutrophil cytoplasmic antigen (ANCA)-associated systemic vasculitis.