Introduction

Primary sclerosing cholangitis (PSC) is a chronic liver disease causing inflammation of the bile ducts, forming progressive strictures and stenoses of bile ducts resulting in recurrent cholangitis, liver cirrhosis and death. Liver transplantation (LT) is necessary for most of the patients as the only curative treatment. However, about 30% of patients develop recurrence of PSC (rPSC), which negatively impacts the outcome of liver grafts and patients. The pathogenesis of recurrent disease is multifactorial, but not entirely understood.

Aims & Methods

The aim of this study was to evaluate the risk factors for rPSC and graft failure (GF) in patients after LT for PSC and PSC/AIH overlap syndrome. Furthermore, we evaluated the patient survival, graft survival and the patient survival after post-LT. In this single-centre retrospective study (IKEM, Prague, Czech Republic), we included patients who underwent LT for PSC in our transplant centre between 1996-2019.

Results

A total of 160 patients after LT for PSC and PSC/AIH overlap syndrome were included in the study. Biliary strictures after LT developed in 67 (41.88%) patients, of which 16 (23.88%) were anastomotic. rPSC was confirmed in totally 51 (31.87%) patients. The mean time to diagnosis of rPSC was 6.2 (CI 95% +- 1.04) years. In multivariant analysis of risk factors for rPSC: Cytomegalovirus (CMV) infection (HR 3.21; 95% CI 1.43-7.19), at least one episode of acute cellular rejection (ACR) (HR 3.00; 95%CI 1.00-9.03) and length of hospitalization after LT (HR 1.03; 95% CI 1.01-1.05) were statistically significant predictors of rPSC.
Liver retransplatation (reLT) due to GF was performed in 23 patients (15.22%). The cause of GF was as follows: rPSC in 18 patients (78.26%), hepatic artery thrombosis in 2 patients (8.69%), primary graft dysfunction in 2 patients (8.69%) and persistent biliary leak in 1 patient (4.35%). The median time to reLT due to rePSC was 8.59 (IQR 6.84,13.81) years. Furthermore, 3 patients required 2nd reLT and 1 patient 3rd reLT. In multivariant survival analysis, CMV infection after LT (HR 5.00; 95%CI 1.22-20.5), PSC/AIH overlap syndrome (HR 6.4; 95% CI 1.13-36.4) and length of hospitalization after LT (HR 1.04; 95%CI 1.01-1.07) were significant predictors for GF. Overall survival of patients in 5 years after LT was 89.5%.

Conclusion

Based on our results, ACR, CMV infection and length of hospitalization post-LT were significant risk factors for rPSC. PSC/AIH overlap syndrome, length of hospitalisation post-LT and CMV infection were associated with worse liver graft outcome.