Delayed intestinal visualization at hepatobiliary scintigraphy is associated with response to long-term treatment with ursodeoxycholic acid in patients with cystic fibrosis-associated liver disease

Background/Aims: Abnormalities of biliary drainage have been documented at hepatobiliary scintigraphy in many but not all patients studied with cystic fibrosis-associated liver disease. Ursodeoxycholic acid was shown to be be neficial in this disease, mainly by improving biliary secretion. Therefore, patients with impaired biliary drainage are expected to obtain the greates t benefit from this treatment. Methods: We evaluated the effects of long-term treatment with ursodeoxychol ic acid in 36 patients with cystic fibrosis-associated liver disease, and c ompared the response in patients presenting a normal (n=18) or delayed time of intestinal visualization (n=18) at baseline hepatobiliary scintigraphy. Results: The mean treatment duration was 58+/-26 (S.D.) months and 63+/-29 months in the groups with normal or delayed time of intestinal visualizatio n, respectively. The time of intestinal visualization decreased (57+23%, p< 0.001) from baseline in patients with initially abnormal values and became normal in four (22%). Treatment failure, i.e. lack of sustained normalizati on of serum liver enzymes or the occurrence of a clinically relevant advers e event, was more frequently observed in patients with a normal time of int estinal visualization at baseline (OR, 5.50; 95% CI, 1.32-22.7). When only clinically relevant adverse events were considered, they occurred in six of the latter patients Giver transplantation in one case, (development of ult rasographic or endoscopic signs of portal hypertension in six cases), but i n only one patient (development of portal hypertension) in the group with d elayed time of intestinal visualization (OR, 10.82; 95% CI, 1.17-100.4). Conclusions: Delayed intestinal visualization at hepatobiliary scintigraphy in patients with cystic fibrosis-associated liver disease seems to predict a better response to ursodeoxycholic acid.