Ursodeoxycholic acid (UDCA) treatment for primary sclerosing cholangitis (PSC) has been considered a rational therapy, though its effectiveness in the clinical course is still open to discussion. In this report, we describe a 22-year-old man with PSC at an early stage, which was associated with ulcerative colitis (UC). He showed progressive strictures of bile ducts over a 1.5-year period in spite of an improvement in the biochemical parameters by UDCA treatment. Therefore, care should be taken in interpreting the effectiveness of UDCA, because the biochemical parameters may not change in parallel with the clinical course of PSC.
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