Abstract

Experimental and clinical observation suggest that patients with primary biliary cirrhosis (PBC) have endothelial dysfunction. Postischemic digital blood flow, nailfold capillaroscopy, von Willebrand factor (vWf) and tissue-type plasminogen activator (t-PA) plasma levels were examined in 59 PBC patients. Forty-six subjects (15 with liver diseases other than PBC, 11 hypercholesterolemics, 20 healthy subjects) served as controls. PBC versus healthy controls (209.8 +/- 1.4% and 16.54 +/- 1.44 ng/ml vs. 120.2 +/- 1.4% and 9.91 +/- 1.49 ng/ml; p<0.001) and related to bilirubin (r = 0.38, p<0.02; r = 0.47, p<0.0005, respectively). vWf was also increased in other liver diseases (249.9 +/- 1.7%; p<0.001) and related to bilirubin (r = 0.59, p<0.05). Postischemic finger blood flow negatively correlated with vWf(p<0.05 or less). Our data indicate that PBC patients have microvascular disease. Whether vessels other than those of the fingers were involved remained unclear. vWf and t-PA might reflect a dysfunction of teh hepatic vascular endothelium.

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