Plasma ADAMTS-13 protein is not associated with portal hypertension or hemodynamic changes in patients with cirrhosis.

BACKGROUND: Activated hepatic stellate cells synthesize the matrix metalloprotease ADAMTS13, which may be involved in the development of liver cirrhosis and portal hypertension. Plasma ADAMTS13 activity has been reported as both increased and decreased in cirrhosis, but ADAMTS13 protein has not previously been examined. AIM: To evaluate ADAMTS13 protein in the hepatic circulation and the relation to disease severity, portal pressure, and systemic hemodynamics in cirrhotic patients. METHODS: Sixty-one cirrhotic patients (Child class: A=22; B=21; C=18) and nine healthy controls underwent a liver vein catheterization with measurement of splanchnic and systemic hemodynamics, and plasma ADAMTS13 protein concentration in a hepatic vein and the femoral artery. RESULTS: ADAMTS13 protein concentrations were increased in cirrhotic patients compared with controls (774ng/ml [IQR: 585-955] vs. 538ng/ml [IQR: 484-631], p<0.03). There were no significant correlations to MELD score, Child Pugh score, portal pressure, nor systemic vascular resistance or cardiac output. CONCLUSIONS: The increased concentration of ADAMTS13 protein in the hepatic circulation may reflect an increased number of active hepatic stellate cells in cirrhosis. However, ADAMTS13 was unrelated to portal hypertension and systemic hemodynamics. In conclusion, ADAMTS13 does not appear to be associated to disease severity or the hemodynamic derangement in patients with cirrhosis.