Mini review
Liver transplantation in PBC and PSC: Indications and disease recurrence

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Summary

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent major indications for liver transplantation (LT). Despite the steady increase in the incidence and prevalence of PBC, the number of liver transplants for PBC has fallen in recent years, whereas the number of transplants for PSC has remained stable. Indications for LT for PBC and PSC are no different from those of other causes of chronic liver disease, apart from some disease-specific indications. PBC and PSC have more favourable outcomes after LT, compared to viral hepatitis and alcohol-associated liver disease. Numerous studies have clearly demonstrated that PBC and PSC recur after LT. The diagnosis of recurrent disease should be made on agreed criteria. The impact of recurrent disease on survival is unclear. Study of recurrent PBC and PSC may provide a better understanding of the mechanisms of these diseases in the native liver.

Introduction

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent major indications for liver transplantation (LT) in western countries [1]. As early mortality after LT has fallen, attention has become focused on long-term outcomes, with recurrent disease emerging as a significant cause of late graft dysfunction and graft failure [2].

Here we review the available data on transplant activity, survival, current indications and characteristics of recurrent disease after LT for PBC and PSC.

Section snippets

Transplant activity

Cholestatic liver disease is becoming an increasingly uncommon indication for LT [3]. Data from United Network for Organ Sharing (UNOS) show that among 2931 cadaveric liver transplants in 1991, 18% (n = 530) were for cholestatic liver disease, compared with 10% (n = 472) of 4579 in 2000 and 7.8% (n = 475) of 6069 in 2008.

For PBC, the UNOS database shows that, between 1995 and 2006 [4], the absolute number of LT increased by mean 249 per year whereas the absolute number of LT performed for PBC

Survival after Liver Transplantation

Cholestatic liver diseases have more favourable outcomes after LT as compared to viral hepatitis and alcohol-associated liver disease [11]. An analysis of the ELTR in 2003 of 2959 patients with PBC showed a 1-, 5-, and 10-year patient and graft survival of 83, 77 and 69% and 79, 71 and 64%, respectively. The 1-, 5-, and 10-year patient and graft survival in 1731 patients with PSC was 83, 75% and 66% and 78, 65 and 54%, respectively [1].

In 2010, Kashyap reported a retrospective analysis of the

Primary biliary cirrhosis

Indications for LT for PBC are not different from those of other etiologies: an unacceptable quality of life because of liver disease or an anticipated survival of 1 year or less. In 1999, Christensen showed that the optimal time for transplantation in PBC (defined as the point when the probability of survival after transplantation is greater than the probability of survival without transplantation) is when the serum bilirubin is around 10 mg/dl (170 mmol/l) [13]. Referral to a LT unit should be

Primary biliary cirrhosis

Recurrent PBC (rPBC) was first reported in 1982 [82], and despite initial controversy, is now widely accepted in both cadaveric and LD grafts [83], [84], [85]. The reported prevalence rates range from 0 to 35% [86], [87], [88], [89], [90], [91], [92], [93], and this wide variability may be due different approaches by transplant units to the use of protocol biopsies and to the variable adherence to histological criteria used in different centers. Data from our center in the period 1982–2002,

Conclusion

PBC and PSC are major indication for LT. Numerous studies have clearly demonstrated recurrence after liver transplant but the underlying pathological mechanism are poorly understood. The diagnosis of recurrent disease should be made on agreed criteria. The impact of recurrent disease on survival is unclear. No effective treatment is available at the present for both diseases, apart from the endoscopic management of bile duct strictures in patients with rPSC.

Further studies are needed to better

Disclosure of interest

The authors have no conflicts of interest to disclose.

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