Recurrence of Primary Sclerosing Cholangitis After Liver Transplantation: A French National Cohort Study Including 571 Patients.

Introduction: Primary sclerosing cholangitis (PSC) may recur after liver transplantation (LT). We aimed to evaluate the incidence of recurrent PSC (rPSC), its characteristics, and risk factors, in a large cohort with long-term follow-up.

Methods: We conducted a nationwide multicenter retrospective study in France, including all adult patients transplanted for PSC from March 1985 to March 2019.

PBC-HOPE: A Randomized Controlled Trial of Hypnosis and Psychoeducation in Women With Primary Biliary Cholangitis and Fatigue.

Introduction: Fatigue is the main symptom of primary biliary cholangitis (PBC) but has not yet been improved by any therapeutic intervention. This study evaluated the efficacy and safety of hypnosis and psychoeducation in improving fatigue associated with PBC (PBC-HOPE ClinicalTrial.gov number, NCT03630718).

Long-Term Efficacy and Safety of Selective PPARδ Agonist Seladelpar in Primary Biliary Cholangitis: ASSURE Interim Study Results.

Introduction: The objective of these analyses was to evaluate interim data from the ongoing, open-label, long-term efficacy and safety ASSURE study of seladelpar, a selective peroxisome proliferator-activated receptor δ agonist, in primary biliary cholangitis.

Methods: Patients rolling over from the phase 3, randomized, placebo-controlled, 12-month RESPONSE study or with previous participation in earlier legacy seladelpar studies were enrolled. Interim evaluations included composite biochemical response (alkaline phosphatase <1.

Obeticholic Acid vs Fibrates as Second-Line Therapy for Primary Biliary Cholangitis: Systematic Review and Meta-Analysis.

Background: Around 40% of patients diagnosed with Primary Biliary Cholangitis (PBC) experience a suboptimal biochemical response to ursodeoxycholic acid (UDCA).

Aims: We aimed to compare fibrates and obeticholic acid (OCA) as add-on second-line therapy and evaluate whether these drugs hold superiority over each other.

Methods: We systematically searched Embase, MEDLINE, and Cochrane CENTRAL for studies comparing fibrates and OCA published by May 6, 2024.

Development of a Question Prompt List for People Living With Primary Biliary Cholangitis: A Delphi Study.

Background And Aims: Clinical practice guidelines support caregivers to manage liver diseases. However, people with lifelong conditions often lack guidance to understand what aspects of care are most important and how their disease should be managed. This study aimed to create a question prompt list (QPL) for individuals with primary biliary cholangitis (PBC) including key questions (directed) to their treating physician that are most likely to improve their outcome.

Current approach to diagnosis and management of low-phospholipid associated cholelithiasis syndrome.

Purpose Of Review: Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare genetic form of intrahepatic cholesterol lithiasis, affecting mainly young adults. This review describes the recent advances in genetic and clinical characterization, diagnosis and management of LPAC syndrome.

Recent Findings: Recent publications report data from several retrospective cohorts.

Type of calcineurin inhibitor and long-term outcomes following liver transplantation in patients with primary biliary cholangitis - an ELTR study.

Background & Aims: Tacrolimus has been associated with recurrence of primary biliary cholangitis (PBC) after liver transplantation (LT), which in turn may reduce survival. This study aimed to assess the association between the type of calcineurin inhibitor used and long-term outcomes following LT in patients with PBC.

Methods: Survival analyses were used to assess the association between immunosuppressive drugs and graft or patient survival among adult patients with PBC in the European Liver Transplant Registry.

Recurrence of autoimmune liver diseases after liver transplantation: Review and expert opinion statement.

Autoimmune liver diseases (AILDs) constitute the fourth most common indication for liver transplantation (LT) across the world. In general, the outcomes after LT are acceptable; however, disease recurrence after LT is common for all AILD, which can negatively affect graft and overall survival. Several questions persist, including the risk factors associated with recurrent disease, optimal antirejection medications, strategies to reduce the risk of recurrence, and how to best incorporate these strategies into clinical practice.

Prognostic scores for ursodeoxycholic acid-treated patients predict graft loss and mortality in recurrent primary biliary cholangitis after liver transplantation.

Background & Aims: Recurrent primary biliary cholangitis (rPBC) develops in approximately 30% of patients and negatively impacts graft and overall patient survival after liver transplantation (LT). There is a lack of data regarding the response rate to ursodeoxycholic acid (UDCA) in rPBC. We evaluated a large, international, multi-center cohort to assess the performance of PBC scores in predicting the risk of graft and overall survival after LT in patients with rPBC.

PPAR agonists in PBC: Where do we go from here? Or how to choose between the new and the old.

The recent phase 3 trials of peroxisome proliferator-activated receptor (PPAR) agonists in primary biliary cholangitis (PBC) patients with incomplete response to ursodeoxycholic acid (UDCA) demonstrated very promising short-term biochemical responses. However, long-term outcomes, crucial in chronic diseases like PBC, remain uncertain. While real-world data (RWD) support surrogate endpoints, there's a need to validate long-term efficacy especially with combination therapies.

Spontaneous perforation of the common bile duct: an uncommon complication of primary sclerosing cholangitis.

Objectives: To describe the MR features and prognosis of patients with an uncommon complication of primary sclerosing cholangitis (PSC) characterized by a spontaneous perforation of the common bile duct (CBD) resulting in a peri-biliary collection and a pseudo-cystic appearance of the CBD.

Methods: A single-center cohort of 263 patients with PSC who had at least two MRIs between 2003 and 2022 and a minimum follow-up of 1 year was retrospectively analyzed. MRI data (characteristics of CBD perforation and MR features of PSC) and clinical data were assessed.

Exploring the impact of ursodeoxycholic acid therapy on COVID-19 in a real-word setting.

Recent data suggest that ursodeoxycholic acid (UDCA) therapy may reduce susceptibility to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and even improve clinical outcomes when coronavirus disease-2019 (COVID-19) was diagnosed. However, clinical evidence of UDCA's ability to prevent severe forms of COVID-19 remains limited and contradictory. We evaluated the association between UDCA exposure and the risk of hospitalization for COVID-19 in a large multicenter population of patients with chronic liver disease (CLD) followed during the pandemic period before vaccination.

Obeticholic acid as a second-line treatment for low phospholipid-associated cholelithiasis syndrome.

Background: Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare genetic cause of hepatolithiasis. A pathogenic variant of the ABCB4 gene is reported in half of all patients. Ursodeoxycholic acid (UDCA) is the only drug approved.

Autoimmune hepatitis recurrence after liver transplantation: "Les jeux sont faits".

Autoimmune hepatitis (AIH) may recur after liver transplantation (LT). The aims of this study were to evaluate the incidence and risk factors for recurrent autoimmune hepatitis (rAIH). A multicenter retrospective French nationwide study, including all patients aged ≥16 transplanted for AIH, with at least 1 liver biopsy 1 year after LT, was conducted between 1985 and 2018.

Adequate versus deep response to ursodeoxycholic acid in primary biliary cholangitis: To what extent and under what conditions is normal alkaline phosphatase level associated with complication-free survival gain?

Background And Aims: Normal alkaline phosphatase (ALP) levels in ursodeoxycholic acid (UDCA)-treated patients with primary biliary cholangitis (PBC) are associated with better long-term outcome. However, second-line therapies are currently recommended only when ALP levels remain above 1.5 times the upper limit of normal (×ULN) after 12-month UDCA.

Optimizing therapy in primary biliary cholangitis: Alkaline phosphatase at six months identifies one-year non-responders and predicts survival.

Background And Aims: Patients with primary biliary cholangitis (PBC) and insufficient response to ursodeoxycholic acid (UDCA), currently assessed after 1 year, are candidates for second-line therapy. The aims of this study are to assess biochemical response pattern and determine the utility of alkaline phosphatase (ALP) at six months as a predictor of insufficient response.

Methods: UDCA-treated patients in the GLOBAL PBC database with available liver biochemistries at one year were included.

Protective potential of the gallbladder in primary sclerosing cholangitis.

Background & Aims: Gallbladder enlargement is common in patients with primary sclerosing cholangitis (PSC). The gallbladder may confer hepatoprotection against bile acid overload, through the sequestration and cholecystohepatic shunt of bile acids. The aim of this study was to assess the potential impact of the gallbladder on disease features and bile acid homeostasis in PSC.

Focus on Liver Function Abnormalities in Patients With Turner Syndrome: Risk Factors and Evaluation of Fibrosis Risk.

Context: Liver function abnormalities (LFAs) have been described in patients with Turner syndrome (TS). Although a high risk of cirrhosis has been reported, there is a need to assess the severity of liver damage in a large cohort of adult patients with TS.

Objective: Evaluate the types of LFAs and their respective prevalence, search for their risk factors, and evaluate the severity of liver impairment by using a noninvasive fibrosis marker.