Recurrence of Primary Sclerosing Cholangitis After Living Donor Liver Transplantation

Sumihito Tamura; Yasuhiko Sugawara; Junichi Kaneko; Yuichi Matsui; Junichi Togashi; Masatoshi Makuuchi


Liver International. 2007;27(1):86-94. 

In This Article

Abstract and Introduction

Background/aims: Cumulative experience in deceased donor liver transplantation for end-stage liver disease due to primary sclerosing cholangitis (PSC) suggests that liver transplantation is the treatment of choice with excellent results. Reports on the outcome of live donor liver transplantation (LDLT) for PSC, however, remain anecdotal.
Methods: The clinical course and genetic disposition of nine patients who underwent LDLT for PSC were analyzed. The median follow-up period was 3.5 years.
Results: Cumulated 5-year patient and graft survival rates were 90% and 71%, respectively. Recurrent PSC was diagnosed in four patients. Ratios of freedom from recurrent PSC at 1, 3, and 5 years were 100%, 73%, and 49%, respectively. The mean time to recurrence was 3.3 years. Excluding the one case with a biologically unrelated donor, recurrent PSC was diagnosed in 50% (4/8). None of the patients presented with the human leukocyte antigen-B8DR3 haplotype, which is associated with a higher susceptibility for developing PSC among the Caucasian population. Overall patient survival of LDLT for PSC seems to equal that of deceased donor liver transplantation.
Conclusions: PSC might recur earlier at a higher ratio after LDLT. Further study with protocol cholangiogram and genetic considerations, including high resolution human leukocyte antigen haplotype analysis, is necessary.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammatory and fibrotic bile duct lesions forming multiple strictures and ectatic dilatations of the intra- and extrahepatic bile ducts.[1,2,3] Experience in Western countries suggests a significant male predominance, with an average onset at approximately 40 years of age, and often complicated by inflammatory bowel disease.[4,5] The disease is progressive, commonly leading to liver failure, and a median survival after diagnosis of 10-12 years.[5,6] In Western countries, PSC is considered to be an important indication for liver transplantation with a 5-year graft survival of approximately 80%.[7,8,9,10] Although the precise etiology and pathogenesis of the disease remain unknown, both immunologic and genetic factors are thought to have a role.[11,12] Recent studies based on deceased donor liver transplantation (DDLT) suggest that PSC can recur.[7,9,13,14,15,16]

Owing to the limited number of organ donations from deceased donors in some Asian countries, live donor liver transplantation (LDLT) is performed as the mainstream treatment for end-stage liver disease, including that resulting from cholestatic disease.[17] There are few reports, however, on the outcome of liver transplantation with the use of partial liver grafts from related living donors for end-stage liver disease due to PSC. Unlike in DDLT, the postoperative course might be affected by the shared genetic disposition between the recipient and the donor, impacting the long-term results. It is not known whether the outcome of related LDLT is equivalent to that of DDLT for PSC. Thus, we retrospectively examined our experience in LDLT for PSC and describe the results herein.


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