Recurrent Primary Sclerosing Cholangitis After Liver Transplantation



Fig. 17.1
PSC recurrence-free survival after transplant (From: Campsen et al. [8])





Living Donor Liver Transplantation


In terms of recurrent PSC after LDLT, there is limited information of about posttransplant outcomes. Data from the A2ALL Study reported that PSC patients had a significantly higher survival rate compared to other disease etiologies [37]. Data from a Japanese survey study on 114 PSC patients all undergoing LDLT at 29 institutions reported a recurrence rate of 27 % and graft loss rates of 69 % in patients with recurrent disease. They reported the following as risk factors for recurrent disease in multivariate analysis: high MELD scores, first-degree-relative donors, postoperative CMV infection, and early biliary anastomotic complications [15]. However, since there is no DDLT comparator group, these data are difficult to place in context. An analysis of the SRTR registry compared patient and graft survival rates for autoimmune and cholestatic disease (including PSC) for LDLT vs. DDLT recipients. There was no difference in patient or graft survival rates for LDLT vs. DDLT in this cohort [26].


Summary Paragraph


An uncommon indication for liver transplantation, PSC is associated with excellent long-term survival rates largely due to the relatively young age of recipients and their absence of comorbid medical conditions which could jeopardize the success of the operation. However, disease recurrence occurs in about one in six patients. The diagnosis may be suspected by elevated liver function tests, typically the alkaline phosphatase or bilirubin, and requires confirmation with either liver biopsy or cholangiography with ERCP or cross-sectional imaging. While numerous risk factors for recurrence have been reported, none have practical implications. There is no known therapy for recurrent disease that predictably changes its natural history. Most patients are administered ursodiol, and symptomatic cholangitis is treated with antibiotics and biliary drainage procedures, as indicated. Recurrent disease can be managed effectively, and graft loss requiring retransplantation does not occur in most cases.


References



1.

Adam R. Evolution of indications and results of liver transplantation in Europe. A report from the European Liver Transplant Registry (ELTR). J Hepatol. 2012;57:675–88.CrossRef


2.

Alabraba E, Nightingale P, Gunson B, Hubscher S, Olliff S, Mirza D, Neuberger J. A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl. 2009;15:330–40.CrossRef


3.

Al-Judaibi B, Hernandez Alejandro R, Uhanova J, Marotta P, Mosli M, Chandok N. Duct-to-duct biliary anastomosis yields similar outcomes to Roux-en-Y hepaticojejunostomy in liver transplantation for primary sclerosing cholangitis. Hepat Mon. 2015;15:e18811.CrossRef


4.

Alexander J, Lord JD, Yeh MM, Cuevas C, Bakthavatsalam R, Kowdley KV. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2008;14:245–51.CrossRef


5.

Azeem N, Tabibian JH, Baron TH, Orhurhu V, Rosen CB, Petersen BT, et al. Use of a single-balloon enteroscope compared with variable-stiffness colonoscopes for endoscopic retrograde cholangiography in liver transplant patients with Roux-en Y biliary anastomosis. Gastrointest Endosco. 2013;77:568–77.CrossRef


6.

Bangarulingam SY, Gossard AA, Petersen BT, Ott BJ, Lindor KD. Complications of endoscopic retrograde cholangiopancreatography in primary sclerosing cholangitis. Am J Gastroenterol. 2009;104:855–60.CrossRef


7.

Brandsaeter B, Schrumpf E, Bentdal O, Brabrand K, Smith HJ, Abildgaard A, et al. Recurrent primary sclerosing cholangitis after liver transplantation: a magnetic resonance cholangiography study with analyses of predictive factors. Liver Transpl. 2005;11:1361–9.CrossRef


8.

Campsen J, Zimmerman MA, Trotter JF, Wachs M, Bak T, Steinberg T, Kam I. Clinically recurrent primary sclerosing cholangitis following liver transplantation: a time course. Liver Transpl. 2008;14:181–5.CrossRef


9.

Cholongitas E, Shusang V, Papatheodoridis GV, Marelli L, Manousou P, Rolando N, et al. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2008;14:138–43.CrossRef


10.

Damrah O, Sharma D, Burroughs A, Rolando N, Fernando B, Davidson B, Rolles K. Duct-to-duct biliary reconstruction in orthotopic liver transplantation for primary sclerosing cholangitis: a viable and safe alternative. Transpl Int. 2012;25:64–8.CrossRef


11.

Distante V, Farouk M, Kurzawinski TR, Ahmed SW, Burroughs AK, Davidson BR, Rolles K. Duct-to-duct biliary reconstruction following liver transplantation for primary sclerosing cholangitis. Transpl Int. 1996;9:126–30.CrossRef

Oct 9, 2017 | Posted by in GASTROENTEROLOGY | Comments Off on Recurrent Primary Sclerosing Cholangitis After Liver Transplantation
Premium Wordpress Themes by UFO Themes