The combination of primary sclerosing cholangitis and CCR5-Δ32 in recipients is strongly associated with the development of nonanastomotic biliary strictures after liver transplantation

Liver Int. 2011 Sep;31(8):1102-9. doi: 10.1111/j.1478-3231.2010.02422.x. Epub 2010 Dec 7.

Abstract

Background: The role of the immune system in the pathogenesis of nonanastomotic biliary strictures (NAS) after orthotopic liver transplantation (OLT) is unclear. A loss-of-function mutation in the CC chemokine receptor 5 (CCR5-Δ32) leads to changes in the immune system, including impaired chemotaxis of regulatory T cells.

Aim: To investigate the impact of the CCR5-Δ32 mutation on the development of NAS.

Methods: In 384 OLTs, we assessed the CCR5 genotype in donors and recipients and correlated this with the occurrence of NAS.

Results: The CCR5-Δ32 allele was found in 65 (16.9%) recipients. The cumulative incidence of NAS at 5 years was 6.5% in wild-type (Wt) recipients vs 17.2% for carriers of the CCR5-Δ32 allele (P<0.01). In recipients with CCR5-Δ32, 50% of all NAS occurred >2 years after OLT, compared with 10% in the Wt group. In multivariate regression analysis, the adjusted risk of developing NAS was four-fold higher in recipients with CCR5-Δ32 (P<0.01). The highest risk of NAS was seen in patients transplanted for primary sclerosing cholangitis (PSC), who also carried CCR5-Δ32 (relative risk 5.4, 95% confidence interval 2.2-12.9; P<0.01). Donor CCR5 genotype had no impact on the occurrence of NAS.

Conclusions: Patients with the CCR5-Δ32 mutation have a four-fold higher risk of developing NAS, compared with Wt recipients. This risk is even higher in patients with CCR5-Δ32 transplanted for PSC. Late development of NAS is significantly more present in patients with CCR5-Δ32. These data suggest that the immune system plays a critical role in the development of NAS after OLT.

MeSH terms

  • Adult
  • Chi-Square Distribution
  • Cholangitis, Sclerosing / complications
  • Cholangitis, Sclerosing / mortality
  • Cholangitis, Sclerosing / surgery*
  • Cholestasis / etiology*
  • Cholestasis / genetics
  • Cholestasis / immunology
  • Cholestasis / mortality
  • Constriction, Pathologic
  • Female
  • Gene Frequency
  • Genetic Predisposition to Disease
  • Graft Survival
  • Humans
  • Immunity, Innate / genetics*
  • Kaplan-Meier Estimate
  • Liver Transplantation / adverse effects*
  • Liver Transplantation / mortality
  • Logistic Models
  • Male
  • Middle Aged
  • Mutation*
  • Netherlands
  • Receptors, CCR5 / genetics*
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Survival Rate
  • Time Factors

Substances

  • Receptors, CCR5