J Korean Soc Transplant.  2013 Mar;27(1):24-28. 10.4285/jkstn.2013.27.1.24.

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

• Lim TS
• Park JY
• Kim SI
• Kang H
• Chung MJ

• KMID: 1488439
• DOI: http://doi.org/10.4285/jkstn.2013.27.1.24

Abstract

Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease. In cases of PSC, liver transplantation is the only effective treatment that can delay the disease's natural course. We report a case of rapidly progressive PSC requiring liver transplantation. A 52-year-old woman visited our hospital with abdominal pain. There was no evidence of PSC, as there was no elevation in cholestatic liver enzymes at her first visit. Although her total bilirubin was in a normal range at the initial visit, liver dysfunction progressed rapidly. Despite endoscopic procedures and ursodeoxycholic acid intake, total bilirubin levels rose to 18.9 mg/dL, and liver transplantation was performed 17 months after her first visit. PSC was pathologically confirmed after liver transplantation.

Keyword

Primary sclerosing cholangitis; Liver transplantation; Ursodeoxycholic acid; Endoscopic retrograde cholangiopancreatography; Magnetic resonance cholangiopancreatography

MeSH Terms

Abdominal Pain
Bilirubin
Cholangiopancreatography, Endoscopic Retrograde
Cholangiopancreatography, Magnetic Resonance
Cholangitis, Sclerosing
Female
Humans
Liver
Liver Diseases
Liver Transplantation
Reference Values
Ursodeoxycholic Acid
Bilirubin
Cholangitis, Sclerosing
Ursodeoxycholic Acid

Figure

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  Fig. 1. (A) Eight months after the initial diagnosis of primary sclerosing cholangitis (PSC), the patient’ s liver biopsy showed a mild degree of lobular and portal activity, and portal fibrosis suggestive of chronic hepatitis (HE stain, ×400). (B) After liver transplantation, which was done 17 months after the initial diagnosis, surgical pathology showed periductal lamellar fibrosis, called “ onion-skin,” a typical finding of PSC (HE stain, ×400).

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  Fig. 2. Twelve months after the initial diagnosis of primary sclerosing cholangitis (PSC), (A) magnetic resonance cholangiopancreatography and (B) endoscopic retrograde cholangiopancreatography findings showed multiple dilatations and strictures of intrahepatic ducts, suggestive of PSC.

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  Fig. 3. The trend of total bilirubin, alkalaine phosphatase, and alanine aminotransferase (ALT) was descri-bed. Abbreviations: PTCS, percuta-neous transhepatic choledochoscopy; ERCP, endoscopic retrograde chol-angiopancreatography; LT, liver tra-nsplantation; ALP, alkaline phosphatase; TB, total bilirubin.

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  Fig. 4. Gross finding of the explanted liver. The liver was mac-ronodular, and diffusely bile stained.

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