Cholangiographic findings in deteriorating primary sclerosing cholangitis

The evolution of primary sclerosing cholangitis (PSC) is difficult to predict, and ERCP may be helpful in diagnosing its complications and treating them. Few descriptions of cholangiographic findings during the course of the disease have yet appeared. We report here on our experience in the management and follow-up by ERCP of six patients with deteriorating PSC.

Between 1981 and 1992, PSC was diagnosed in 18 patients. Clinical, biochemical, and cholangiographic findings were recorded during the follow-up period. An initial group of 12 patients had a good clinical and biochemical course (mean 56 months) and did not require systematic control ERCPs. A second group of six patients, showing cholangiographic features similar to those of the first group at presentation, developed clinical and biochemical deterioration within a mean period of 43 months (range 6-96).

These six patients had a significant elevation of serum bilirubin and at ERCP showed worsening bile duct lesions, with marked strictures and a subsequent increase in the maximum duct diameter in both the extrahepatic and intrahepatic ducts compared to the initial ERCPs. In two of these six patients, the strictures were due to a superimposed cholangiocarcinoma. Endoscopic drainage was unsuccessful, transplantation impracticable due to extrahepatic spread, and both patients died three and five months, respectively, after diagnosis of cholangiocarcinoma. By contrast, four patients with dominant extrahepatic duct involvement benefited from endoscopic procedures (mean follow-up 19 months) without any recurrence of cholangitis and without requiring long-term antimicrobial treatment. The follow-up checks included regular ERCPs performed every six months for duct and stent assessment, and cytology brushing.

Since there are no effective means of early and accurate detection of cholangiocarcinoma, the use of ERCP to compare the course of cholangiographic lesions over time may be of interest. Patients with extrahepatic duct involvement may benefit from endoscopic therapy. By contrast, in patients with dominant intrahepatic duct disease and a deteriorating clinical status, attempts at endoscopic therapy are not helpful, and liver transplantation needs to be considered promptly.