Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival (original) (raw)
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CMAJ open, 2018
rimary biliary cholangitis (PBC) is a rare chronic autoimmune cholestatic liver disease characterized by destruction of the small intrahepatic bile ducts. It predominantly affects middle-aged and elderly women. 1,2 Despite its rarity, PBC is an important cause of liver-related morbidity. 2-5 In the United States, the annual economic burden of PBC has been estimated to be 69−69-69−115 million. 3 There is concern that the economic impact of PBC could increase significantly in the future because of the increasing incidence and prevalence of this disease; 6-12 however, with improved treatment options, the costs may be attenuated. 13-15 The epidemiology of PBC was first described as early as 40 years ago, 16 although decades later no study has yet investigated the national prevalence of PBC in Canada. A number of studies have examined the epidemiology of PBC globally, illustrating considerable variability in the prevalence of the disease; prevalence rates range from 6.7 to 402 cases per million, while incidence rates range from 0.7 to 58 cases per million. 1 A growing number of studies suggest that the incidence and prevalence of PBC are increasing. 6-12 The driver of this growth remains unclear, but it may be the result of an increase in incidence, longer survival and
Transplantation direct, 2017
Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease, with liver transplantation being the sole life-saving treatment for end-stage PSC-related liver disease. However, recurrence of PSC after liver transplantation is a common complication, with the risk factors for recurrence being controversial. We conducted a retrospective chart review of 45 patients who had undergone liver transplantation for PSC at our institute. The risk factors for PSC recurrence and graft failure after liver transplantation were analyzed. The graft survival rates were 55.4% at 5 years and 32.8% at 10 years after liver transplantation for PSC. PSC recurrence was diagnosed in 16 (40%) of 40 patients, at a median 30 months (range, 9-70 months) after liver transplantation. The cumulative incidence rate of PSC recurrence was 24.5% at 3 years, 39.3% at 5 years, and 45.8% at 6 years. Among the 16 patients diagnosed with PSC recurrence, the graft survival rate was 56.3% at 5 years, and 21.9...
OBM Transplantation, 2019
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are idiopathic and progressive autoimmune hepatobiliary disorders that lead to liver failure and a need for liver transplantation in a proportion of individuals with poorly controlled disease. It is currently thought that an environmental agent triggers disease in a genetically susceptible host and to date, xenobiotics, bacteria and a human betaretrovirus have all been linked with PBC. However, there is no consensus on which agents predominates. These disease processes are poorly understood and there are disparate hypotheses concerning the pathogenesis. One theory suggests that the disease is mediated by autoimmunity, whereas others have speculated that they are infectious disease processes that only manifest in individuals with diminished immunity. Clinically, the triggers of disease are difficult to study because of the indolent onset and chronic nature of the disorders. Notably, observations from liver transplantation provide a unique insight into the development of PBC and PSC. Both biliary disorders may reoccur in up to 30%-50% of patients following liver transplantation and many of the factors that influence recurrence have been well described. Prior to transplantation, immunosuppression is not routinely used to treat PBC and PSC because specific treatments have not been shown to have utility or have caused undue side effects. Following
Liver transplantation for primary sclerosing cholangitis
Journal of Hepato-Biliary-Pancreatic Surgery, 1999
and timing of liver transplantation remain as major problems. Data from PSC and control patients listed for liver transplantation from 1990 through 2000 in the Nordic countries were recorded prospectively. Outcomes from the waiting list and after transplantation have been recorded for both groups. For PSC patients, regression analyses have been performed to analyze predictors of outcome. A total of 255 PSC and 610 control patients were accepted on the liver transplantation waiting list from 1990 to 2000. In the PSC group, 223 patients (87%) received a first liver allograft, and 32 patients (13%) died without transplantation. The corresponding figures for the control group were 89% and 10%. For PSC patients, the 5-and 10-year survival from the time of acceptance was 68% and 58%, respectively. A higher Model for End-Stage Liver Disease score and a shorter duration of PSC predicted death on the waiting list for PSC patients. PSC is a frequent indication for liver transplantation. In our material, serum bilirubin or Model for End-Stage Liver Disease score and PSC duration are predictors of outcome including survival of the waiting list. (Liver Transpl 2003; 9:961-969.)
Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation
Liver Transplantation, 2008
Liver transplantation (LT) is the only therapeutic option for end-stage primary sclerosing cholangitis (PSC), but PSC can recur (rPSC) in some patients after LT. The aim of our study was to evaluate the risk factors associated with rPSC. Between 1989 and 2004, 69 patients receiving transplantation for PSC (42 male, mean age 41.9 yr). Clinical and laboratory data, activity/extension and treatment of ulcerative colitis (UC), post-LT cytomegalovirus (CMV) infection, and immunosuppression were evaluated. Determination of rPSC was made by radiological and histological findings. Exclusion criteria were ABO blood group incompatibility, hepatic artery stenosis, and biliary strictures occurring in <3 months post-LT. A total of 48 (70%) patients had PSC and UC pre-LT. rPSC occurred in 7 of 53 (13.5%, 2 patients with de novo UC) who were alive 1 yr after LT and/or met inclusion/exclusion criteria: median 60 (4-120) months. No patient without post-LT UC had rPSC: 0 of 20 vs. 7 of 26 with post-LT UC (P = 0.027). The multivariate logistic regression analysis showed that maintenance steroids for UC (>3 months) post-LT was the only risk factor significantly associated with rPSC (P = 0.025). In conclusion, the presence of UC post-LT, and the need for maintenance steroids post-LT, which is an independent factor, are associated with rPSC. These findings could help elucidate a possible mechanism of PSC pathogenesis. Liver Transpl, 2007. © 2007 AASLD.
Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
Journal of Clinical Medicine
Primary sclerosing cholangitis (PSC) is characterized by inflammation of the whole bile duct system. Liver transplantation is only approved as a curative treatment when it comes to end-stage liver disease. The aim of our study was to assess morbidity, survival rates and PSC recurrence and the impact of donor characteristics in long-term follow-up. This was an IRB-approved retrospective study. A total of 82 patients were identified who were transplanted between January 2010 and December 2021 for PSC. Among these patients, 76 adult liver transplant PSC patients and their corresponding donors were analyzed. Three pediatric cases and three adult patients with a follow-up within <1 year were excluded from further analysis. Median (range) age was 47 years (18–70) with a median (range) lab-MELD of 16 (7–40). Median (range) ICU and hospital stays were 4.6 days (0–147) and 21 days (1–176), respectively. The majority of patients suffered from Crohn’s disease or ulcerative colitis as a conc...
Alimentary Pharmacology & Therapeutics
Background: The importance of primary biliary cholangitis as an indication for liver transplantation has probably been influenced by the introduction of therapies, and changes in selection criteria and disease epidemiology. Aims: To assess the time trends in liver transplantation for primary biliary cholangitis and to evaluate the characteristics of the patient population during the past three decades. Methods: Patients undergoing liver transplantation from 1986 to 2015 in centres reporting to the European Liver Transplantation Registry were included. We excluded combined organ transplantations and patients <18 years. Trends were assessed using linear regression models. Results: We included 112 874 patients, of whom 6029 (5.3%) had primary biliary cholangitis. After an initial increase in the first decade, the annual number of liver transplantation for primary biliary cholangitis remained stable at around 200. The proportion of liver transplantations for primary biliary cholangitis decreased from 20% in 1986 to 4% in 2015 (P < 0.001). Primary biliary cholangitis was the only indication showing a consistent proportional decrease throughout all decades. From the first to the third decade, the age at liver transplantation increased from 54 (IQR 47-59) to 56 years (IQR 48-62) and the proportion of males increased from 11% to 15% (both P < 0.001). Conclusions: We have found a proportional decrease in primary biliary cholangitis as indication for liver transplantation. However, despite treatment with ursodeoxycholic acid and improved disease awareness, the absolute annual number of liver transplantations has stabilised.