Primary Sclerosing Cholangitis: Report of a Case with a Clinical Analysis of the Cases Reported in the Korean Literature

Seo, Jung Kun; Chung, Jun Pyo; Cho, Hyeon Geun; Lee, Kwi Soon; Lee, Kwan Sik; Chon, Chae Yoon; Kang, Jin Kyung; Park, In Suh; Kim, Ki Whang; Kim, Ho Geun; Lee, Sang In

Abstract

We experienced a case of primary sclerosing cholangitis(PSC) in a 40-year-old female who complained of jaundice and pruritus. Marked elevation of serum alkaline phophatase level, typical beaded appearance and pruned-tree appearance on endoscopic retrograde cholangiography, together with a finding of chronic obliterative fibrosing cholangitis on sono-guided gun biopsy specimen of the liver led to a confirmative diagnosis of PSC. The patient responded to ursodeoxycholic acid (UDCA), but was reluctant to treatment and died of hepatic failure 7 months later. PSC is a very rare disease in Korea. So far, only 5 cases including our present case have been reported in the Korean literature. Male-to-female ratio was 2:3 and the median age was 40(27-80 years old). Ulcerative colitis was associated in one case. Four cases involved both intra, and extrahepatic bile ducts and one case was reported to be confined in the intrahepatic bile ducts. Fatality was in 3 cases, 20 days, 36 days, and 7 months after the initial presentaion, respectively. The causes of death were acute cholangitis and sepsis in two, and hepatic failure in one. We herein report a case of PSC and clinical charateristics of the reported cases in Korea, and review the literature with an emphasis on UDCA treatment in PSC.