KDIGO 2025 ADPKD Guideline: A commentary on diagnosis and management of hepatopancreatic manifestations by the ERA Working Group Genes & Kidney.

The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a long-awaited development and the first KDIGO guideline addressing a genetic kidney disease. The approval of tolvaptan as targeted therapy for ADPKD in 2015, marked a pivotal advancement, and galvanized towards a structured approach in research and clinical care. Considering the systemic nature of ADPKD guidance for the management of extrarenal manifestations is of special importance for nephrologists and requires interdisciplinary collaboration.

Liver transplantation indications and strategies in polycystic liver disease: A european survey.

Background Aims: Liver transplantation (LT) remains the only cure for severe polycystic liver disease (PLD). However, LT indications and strategies vary across Europe, resulting in (unwanted) practice variation and unequal access to care for patients with PLD. This study aimed to (1) identify existing PLD-specific LT criteria across European countries, (2) assess which criteria are considered relevant by specialist, and (3) map the variation in liver-kidney transplant strategies among patients with autosomal dominant polycystic kidney disease (ADPKD).

Early Prognostic Factors for Reduced Survival in Autoimmune Hepatitis: A Systematic Review With Meta-analysis.

Background & Aims: Autoimmune hepatitis (AIH) is a severe, life-threatening condition for some patients. This systematic review aimed to identify and rank early prognostic factors associated with reduced survival in AIH.

Methods: A systematic search was conducted in Ovid Medline and embase.

Adherence to United European Gastroenterology Guidelines on Diagnosis and Therapy of Immunoglobulin-G4-Related Digestive Disease.

Introduction: United European Gastroenterology (UEG) Guidelines on immunoglobulin G4 (IgG4)-related digestive disease provides evidence-based recommendations for the diagnosis and management of IgG4-related digestive disease. The aim of this study is to evaluate the adherence to recommendations of this IgG4 guideline across centers in Europe.

Patients And Methods: Questionnaire-based data related to organ involvement, diagnosis, treatment and follow-up of newly diagnosed patients with IgG4-related digestive diseases over a 3-year period, were collected from 14 centers in 11 European countries.

Facilitators and barriers for implementation of a decentralized hepatitis C care chain for people who use drugs in Dutch addiction care.

Background: People who use drugs (PWUD) are an important target population for HCV elimination goals of the World Health Organization (WHO). Case-finding and treatment of these patients is challenging due to population specific barriers like stigma and care avoidance. Low threshold HCV screening and treatment within addiction care may facilitate viral elimination.

Prognostic Value of the TLM3 Biomarker Panel for Early Fibrosis Development in MASLD Within the General Population.

Background & Aims: Fibrotic MASLD is associated with increased morbidity and mortality, often remaining asymptomatic until advanced stages of disease. Predicting fibrosis onset and progression would improve risk stratification and treatment allocation. This study aims to investigate whether a previously identified fibrosis biomarker panel for active fibrogenesis (TLM3) can serve as a prognostic marker panel for fibrosis development in a population at cardiometabolic risk of fibrotic MASLD.

Endoscopic retrograde cholangiopancreatography discharge tool combined with rapid trypsinogen-2 test to predict same-day discharge: a prospective cohort study.

Objectives: Identifying patients at high-risk for endoscopic retrograde cholangiopancreatography (ERCP)-related adverse events (AEs) is important for postendoscopic discharge management. This study assesses two strategies, a urinary trypsinogen-2 (UT-2) dipstick combined with a risk-factor-based ERCP discharge tool, for identifying patients at increased risk of developing AEs.

Methods: Between August 2018 and March 2021, 268 patients were enrolled in a multicenter prospective cohort.

Development and validation of a clinical decision tool for predicting long-term pain reduction following laparoscopic cholecystectomy in patients with symptomatic cholecystolithiasis: a prospective cohort study.

Background: The applicability of the recently reported predictors to predict pain reduction after laparoscopic cholecystectomy (LC) is limited by only predicting short-term outcomes. The aim of this study was to develop and externally validate a model to predict clinically relevant pain reduction 5 years after LC.

Materials And Methods: This study reports on 5-year follow-up data of two multicenter prospective trials in 25 hospitals.

Rifaximin-α in Patients With Recurrent Episodes of Hepatic Encephalopathy Due to Cirrhosis Reduces Healthcare Utilization.

Background And Aims: Hepatic encephalopathy is a frequent complication of cirrhosis. Rifaximin-α has been included in guidelines for secondary prevention of hepatic encephalopathy, but there are few real-world data on its efficacy and impact on healthcare utilization. In this study, we aimed to assess the effect of rifaximin-α on healthcare utilization.

Hepatic Encephalopathy and MELD-Na Predict Treatment Benefit in Autoimmune Hepatitis-related Decompensated Cirrhosis.

Background & Aims: Management of patients with autoimmune hepatitis (AIH)-related decompensated cirrhosis is challenging because of the risk of treatment-related complications and lack of clinical recommendations. We investigated the predictive factors for treatment benefit in AIH-related decompensated cirrhosis at diagnosis and developed an algorithm to guide treatment decisions in clinical practice.

Methods: This retrospective, international, multicenter study included 232 patients with histologically confirmed AIH-related decompensated cirrhosis at diagnosis.

Hepatic Safety Considerations in the Use of Ulipristal Acetate for Symptomatic Uterine Fibroids.

Introduction: Ulipristal acetate (UPA, 5 mg) demonstrated efficacy in symptom reduction for patients with symptomatic fibroids. While registration and post-marketing trials assessing UPA identified few hepatic concerns, post-marketing concerns about potential drug-induced liver injury (DILI) led to significant restrictions, including indication restriction, warning labels and mandatory liver function monitoring. These measures, along with two marketing suspensions, resulted in a decline in UPA use, ultimately leading to the withdrawal of its marketing authorization previously in Canada, Australia, as well as Singapore and in 2024, at the request of the marketing authorization holder for commercial reasons, also for the European Union.

Health-related quality of life is impaired in people with autoimmune hepatitis: Results of a multicentre cross-sectional study within the European Reference Network.

Background And Aims: Impaired health-related quality of life (HRQoL) contributes to the overall disease burden in autoimmune hepatitis (AIH). This study aimed to evaluate HRQoL in people with AIH and to identify potentially modifiable factors associated with impaired HRQoL using validated patient-reported outcome measures.

Approach And Results: Adult patients with AIH diagnosed at 12 European centers were enrolled in this prospective, cross-sectional study from July 2020 to June 2023.

KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary.

The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations.

Exploring the innate immune response in polycystic liver disease.

Rationale: The role of the innate immune system in polycystic liver disease (PLD) has been underexplored despite its potential importance in disease progression. This study explores the innate immune response in PLD patients by analyzing cytokine production of peripheral blood mononuclear cells (PBMCs) in response to various pathogens compared to healthy controls.

Methods: Samples were collected from patients with ADPLD or ADPKD and PLD.

Liver cyst penetration of antibiotics at the target site of infection: a randomized pharmacokinetic trial.

Background: The EASL cystic liver disease guideline states that drug penetration at the site of infection (liver cyst) is essential for successful treatment, but pharmacokinetic (PK) data on cyst penetration are limited.

Objectives: This study aims to investigate tissue penetration of four antibiotics in non-infected liver cysts and explores influencing factors.

Methods: We performed a prospective, randomized single-dose PK-study.

Preventive Measures and Risk Factors for Post-ERCP Pancreatitis: A Systematic Review and Individual Patient Data Meta-Analysis.

Background: Post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) is the most common complication of ERCP, with limited studies comparing combined prophylactic measures and their efficacy relative to individual patient risk profiles. This study aims to perform an individual patient data meta-analysis (IPDMA) to evaluate the contribution of patient and ERCP-related risk factors to PEP development and to identify the best prophylaxis strategies according to the patient's risk profile.

Methods: We systematically searched MEDLINE, Embase, and Cochrane databases until November 2022 for randomized controlled PEP prophylaxis trials.

Gastrointestinal Angiodysplasia Resolution After Transcatheter Aortic Valve Implantation.

Importance: Heyde syndrome is the cooccurrence of aortic stenosis and gastrointestinal bleeding secondary to vascular lesions, including angiodysplasias. Several studies have demonstrated cessation of gastrointestinal bleeding after transcatheter aortic valve implantation (TAVI), but the etiology and effects on vascular lesions are largely unknown.

Objective: To examine the associations of TAVI with gastrointestinal vascular lesions and identify factors associated with recovery among patients with iron deficiency anemia and severe aortic stenosis.

Efficacy and safety of infliximab in patients with autoimmune hepatitis.

Background And Aims: A limited number of drugs are used as standard or alternative therapies in autoimmune hepatitis (AIH). No specific recommendations are available for patients failing to respond to these therapies. We analyzed the efficacy and safety of infliximab in patients with AIH.

Restrictive Strategy vs Usual Care for Cholecystectomy in Patients With Abdominal Pain and Gallstones: 5-Year Follow-Up of the SECURE Randomized Clinical Trial.

Importance: The 1-year results of the SECURE trial, a randomized trial comparing a restrictive strategy vs usual care for select patients with symptomatic cholelithiasis for cholecystectomy, resulted in a significantly lower operation rate after restrictive strategy. However, a restrictive strategy did not result in more pain-free patients at 1 year.

Objective: To gauge pain level and determine the proportion of pain-free patients, operation rate, and biliary and surgical complications at the 5-year follow-up.

Efficacy and safety of palliative treatment in patients with autoimmune liver disease-associated hepatocellular carcinoma.

Introduction And Objectives: Autoimmune liver diseases (AILD) are rare causes hepatocellular carcinoma (HCC), and data on the efficacy and tolerability of anti-tumor therapies are scarce. This pan-European study aimed to assess outcomes in AILD-HCC patients treated with tyrosine kinase inhibitors (TKIs) or transarterial chemoembolization (TACE) compared with patients with more common HCC etiologies, including viral, alcoholic or non-alcoholic fatty liver disease.

Materials And Methods: 107 patients with HCC-AILD (AIH:55; PBC:52) treated at 13 European centres between 1996 and 2020 were included.

Incidence and prevalence of primary biliary cholangitis in the Netherlands - A nationwide cohort study.

Background & Aims: Although primary biliary cholangitis (PBC) is considered a rare disorder, accurate determination of its incidence and prevalence remains challenging due to limited comprehensive population-based registries. We aimed to assess the incidence and prevalence of PBC in the Netherlands over time through the nationwide Dutch PBC Cohort Study (DPCS).

Methods: DPCS retrospectively included every identifiable patient with PBC in the Netherlands from 1990 onwards in all 71 Dutch hospitals.