Vascular impairment in patients with primary biliary cirrhosis.

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.