[Transendoscopic retrograde cholangiography in the diagnosis of primary sclerosing cholangitis. Study of 6 cases].

Primary sclerosing cholangitis (PSC) is a rare disease of unknown etiology characterized by severe chronic inflammation, fibrosis and stenosis of variable length of the extra and/or intrahepatic biliary ducts in the absence of recent operative trauma, biliary stones, cancer or infection. Diagnosis has been made only at operation. The introduction of endoscopic retrograde cholangiography (ERC) offers the possibility of preoperative diagnosis. Six cases of PSC diagnosed by ERC are presented. Characteristic roentgenologic findings include strictures of variable length of extra and intrahepatic biliary ducts, beaded appearance and decreased arborization of intrahepatic biliary tree. The roentgenological anatomy of biliary tree at ERC influence the subsequent therapy, i.e. surgical therapy is indicated if a drainage can be performed above the site of the stenosis, while medical therapy (steroids and/or immunosuppressive drugs) is the choice when intrahepatic biliary tree is involved.