von Willebrand factor antigen and plasminogen activator inhibitor in giant cell arteritis

Article Abstract:

Giant cell arteritis is an inflammatory disease of blood vessels, which features inflammatory lesions of larger arteries caused by granulomas (tumors composed of granular white blood cells). This may be characterized by fever, weight loss, and joint and muscle pains. The temporal artery (at the side of the skull) may be involved in some patients, causing localized or widespread head pain. Tissue tests (biopsies) are not always positive for the disease, so that diagnosis must rely on the clinical symptoms. A laboratory test for the disease would be helpful in such cases. Endothelial cells line the walls of blood vessels and synthesize many important proteins. Damaged endothelial cells secrete von Willebrand factor (which is measured according to the presence of antigen, vWF:Ag, in an antibody-based test), and this is important in allowing platelets to support the clotting process. Plasminogen activator inhibitor is also synthesized in endothelial cells and has the effect of enhancing the stability of clots by inhibiting fibrinolysis (clot breakdown). Levels of vWF:Ag may be abnormally high in patients with giant cell arteritis, which can be accompanied by a reduction in fibrinolysis. The levels of these two endothelium-derived proteins in 63 patients (51 female) with giant cell arteritis were measured. Levels of vWF:Ag were significantly higher in patients than in the general population, and tended to fall with steroid treatment. However, some patients had normal vWF:Ag levels, and levels did not correlate with disease severity. Plasminogen activator inhibitor activity did not vary significantly between patients and healthy subjects and changed only slightly with time. The study indicates that although vWF:Ag was increased in patients with giant cell arteritis, its levels are not useful in diagnosis of the disease due to wide variability. (Consumer Summary produced by Reliance Medical Information, Inc.)

Giant cell arteritis, Temporal arteritis, Antifibrinolytic agents

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Computer based quantitative analysis of capillary abnormalities in systemic sclerosis and its relation to plasma concentration of von Willebrand factor

Article Abstract:

A computerized system that evaluates vessel patterns and measurements may prove useful in assessing patients with small vessel diseases. Researchers photographed the transparent skin layer at the base of the fourth finger of 12 patients with limited cutaneous systemic sclerosis (lSSc), 5 patients with diffuse systemic sclerosis (dSSc), and 17 healthy volunteers (controls) for evidence of vessel abnormalities. They also measured blood levels of the von Willebrand factor (VWF) in all participants and the finger pressure of 16 patients with vessel disease. Computerized analysis of the photograph tracings showed that patients with vessel disease had fewer vessels per area measured and larger vessel loops than the controls. Eleven of 16 patients had finger pressure measurements indicating artery abnormalities and also had photograph analyses that were out of range. There was no significant association between VWF blood levels and any of the vessel measurements.

Abnormalities, Capillaries, Von Willebrand's disease

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Increased release of von Willebrand factor antigen from endothelial cells by anti-DNA autoantibodies

Article Abstract:

An immune response to a patient's own DNA may play a role in initiating endothelial tissue injury in patients with systemic lupus erythematosus (SLE). Endothelial cells are cells that line blood vessels and major organs of the body. Researchers isolated the immune protein, IgG, from blood samples of 17 patients with SLE and 9 healthy volunteers (controls) and studied the interaction of IgG-treated endothelial cells with antibodies to these cells. IgG-treated endothelial cells from the patients with SLE had a greater tendency to bind to the antibodies than the controls as well as expressing the von Willebrand factor. This binding tendency was even greater in IgG-treated cells as compared to IgG-treated cells not containing the antibody to DNA.

Systemic lupus erythematosus

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