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. 2021 Aug;34(8):1455-1467.
doi: 10.1111/tri.13925. Epub 2021 Jun 28.

Recurrence of primary sclerosing cholangitis after liver transplantation - analysing the European Liver Transplant Registry and beyond

Thijmen Visseren  1   2 , Nicole Stephanie Erler  3   4 , Wojciech Grzegorz Polak  2 , René Adam  5 , Vincent Karam  5 , Florian Wolfgang Rudolf Vondran  6 , Bo-Goran Ericzon  7 , Douglas Thorburn  8 , Jan Nicolaas Maria IJzermans  2 , Andreas Paul  9 , Frans van der Heide  10 , Pavel Taimr  11 , Petr Nemec  12 , Jacques Pirenne  13 , Renato Romagnoli  14 , Herold Johnny Metselaar  1 , Sarwa Darwish Murad  1 ; European Liver and Intestine Transplantation Association (ELITA)
Collaborators, Affiliations

Recurrence of primary sclerosing cholangitis after liver transplantation - analysing the European Liver Transplant Registry and beyond

Thijmen Visseren et al. Transpl Int. 2021 Aug.

Abstract

Liver transplantation for primary sclerosing cholangitis (PSC) can be complicated by recurrence of PSC (rPSC). This may compromise graft survival but the effect on patient survival is less clear. We investigated the effect of post-transplant rPSC on graft and patient survival in a large European cohort. Registry data from the European Liver Transplant Registry regarding all first transplants for PSC between 1980 and 2015 were supplemented with detailed data on rPSC from 48 out of 138 contributing transplant centres, involving 1,549 patients. Bayesian proportional hazards models were used to investigate the impact of rPSC and other covariates on patient and graft survival. Recurrence of PSC was diagnosed in 259 patients (16.7%) after a median follow-up of 5.0 years (quantile 2.5%-97.5%: 0.4-18.5), with a significant negative impact on both graft (HR 6.7; 95% CI 4.9-9.1) and patient survival (HR 2.3; 95% CI 1.5-3.3). Patients with rPSC underwent significantly more re-transplants than those without rPSC (OR 3.6, 95% CI 2.7-4.8). PSC recurrence has a negative impact on both graft and patient survival, independent of transplant-related covariates. Recurrence of PSC leads to higher number of re-transplantations and a 33% decrease in 10-year graft survival.

Keywords: bayesian statistics; disease recurrence; liver transplantation; patient and graft survival; primary sclerosing cholangitis.

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Conflict of interest statement

The authors who have taken part in this study declared that they do not have any conflict of interest with respect to this manuscript. Dr. Thorburn reports personal fees from Intercept, Falk Pharma, Mirum, Cymabay, and Engitix, all outside the submitted work.

Figures

Figure 1
Figure 1
Flow chart of the 1,573 patients transplanted for PSC. The flow chart shows the number of patients (n = 1,573) for which additional data were provided by 48 transplant centres, patients who were excluded (n = 24), and the number of patients used for patient (n = 1,428) and graft (n = 1,336) survival analyses.
Figure 2
Figure 2
Flowchart of the 1,879 transplants performed in 1,549 patients. The flowchart shows the number of first, second, third and fourth transplants performed. The rPSC cases are displayed throughout the chart as nrPSC, and the outcomes are shown stratified by cause; liver related (liver rel.), nonliver related (nonliver rel.), and unknown.
Figure 3
Figure 3
Kaplan–Meier survival curves for patient and graft survival, including all re‐transplants.
Figure 4
Figure 4
Expected patient survival with and without rPSC. Expected patient survival and corresponding 95% CIs in scenarios with and without rPSC. The curves show the scenarios when rPSC is diagnosed after 90 days (a), 5 years (b) and 10 years (c). The effect of rPSC is more detrimental when diagnosed early after LT, compared with a later onset.
Figure 5
Figure 5
Expected patient survival with and without rPSC, in scenarios with and without re‐transplants. Expected patient survival and corresponding 95% CIs in different scenarios of rPSC and re‐transplants. Panel a: estimated survival declines faster after rPSC. Panel b: re‐transplant does not affect the decline in estimated survival in patients with rPSC, but results in steeper decline for patients without rPSC.
Figure 6
Figure 6
Expected graft survival with and without rPSC. Expected survival of first grafts and corresponding 95% CIs in different scenarios with and without rPSC. The curves show the scenarios when rPSC is diagnosed after 90 days (a), 5 years (b) and 10 years (c). The effect of rPSC is detrimental for graft survival irrespective of timing of recurrence.
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