• Yuji Fujita, Takeshi Sugaya, Ayano Inui, and Shigemi Yoshihara.
• Department of Pediatrics, Dokkyo Medical University.
• Tohoku J. Exp. Med. 2021 Feb 1; 253 (2): 109-112.

AbstractPrimary sclerosing cholangitis is a rare disease with poor prognosis that potentially leads to liver cirrhosis and is often complicated by inflammatory bowel disease. Although ursodeoxycholic acid is the most commonly used drug to treat primary sclerosing cholangitis, its effectiveness in treating primary sclerosing cholangitis has not yet been established. An 11-year-old girl had a fever, upper and lower abdominal pain, and bloody stools. Colonoscopy revealed ulcerative colitis. She also had elevated hepatobiliary enzyme levels and C-reactive protein levels, indicating cholangitis after starting food intake, and primary sclerosing cholangitis was diagnosed with endoscopic retrograde cholangiography. Her hepatobiliary enzyme levels gradually improved after ursodeoxycholic acid was started, and symptoms did not recur after food intake. Primary sclerosing cholangitis should be considered if patients, even children, with inflammatory bowel disease, have upper abdominal pain with elevated biliary enzyme levels. The clinical guidelines for primary sclerosing cholangitis treatment have recommended that ursodeoxycholic acid should not be actively used. However, there are some recent reports stating its effectiveness for primary sclerosing cholangitis. In this patient, ursodeoxycholic acid may have been effective for the normalization of the hepatobiliary enzymes. However, it is unknown whether ursodeoxycholic acid improves long-term prognosis. Hence, further evidence regarding the effectiveness of ursodeoxycholic acid in the treatment of primary sclerosing cholangitis needs to be established.

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