Follicular cholangitis (FC) is a rare, benign inflammatory biliary disorder that can closely mimic malignancy. We present the case of a 34-year-old lady with an incidentally detected distal common bile duct (CBD) lesion. Preoperative imaging was suggestive of periampullary malignancy. However, serology and biopsy findings were inconclusive. Intraoperative frozen section analysis revealed cholangitis with prominent lymphoid aggregates, ruling out malignancy. These critical intraoperative...

CONCLUSIONS: FC levels did not significantly differ between PSC-IBD and IBD groups. The available evidence is limited and heterogeneous. Larger, well-designed studies are needed to determine whether FC can serve as a surrogate biomarker of PSC progression, particularly in patients with endoscopic remission of colitis or without concomitant IBD.

CONCLUSION: This study provides genetic evidence for the comorbidity of IBD and PSC, enhancing our understanding of the pathophysiological aspects of both diseases.

CONCLUSION: PC enables direct visual inspection of the biliary tree with correlative cholangiography to better appreciate and manage biliary pathology not amenable to peroral cholangioscopy, ERCP, or MRCP.

BACKGROUND: Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of inflammatory bowel disease (IBD). No therapy currently halts disease progression. The strong gut-liver axis implicated in PSC pathogenesis supports the investigation of microbiome-targeted treatments. Oral vancomycin (OV), an antibiotic with potential immunomodulatory properties, has shown encouraging results in improving clinical symptoms and liver biochemistry in PSC. However, prospective data on...

CONCLUSION: These findings identify BTN3A2 as a causal protective factor in PSC, mediated by DNA methylation and CD14+CD16+ monocyte-driven immunity, highlighting its therapeutic potential for precision medicine.

CONCLUSIONS: This multicenter study identified LDLT, acute cellular rejection, IBD, and biliary complications as independent risk factors for rPSC. These findings underscore the need for individualized post-transplant surveillance and provide important considerations for graft selection and perioperative management in patients with PSC, particularly in settings where LDLT is predominant.

CONCLUSION: This study confirms the prognostic value of quantitative MRCP (number of strictures and proportion of 3-5 mm diameter ducts) on long-term transplant-free survival in PSC and warrants further study on incorporating quantitative MRCP metrics into existing prognostic risk models.

CONCLUSION: MRCP+ metrics have clinical utility to support patient management alongside addressing key gaps, including standardising MRCP assessment, early detection of disease, and quantification of risk.

The care of patients with cholestatic liver diseases such as primary sclerosing cholangitis (PSC) is challenging, partly due to the lack of knowledge of disease pathogenesis and suitable in vitro models for disease modeling and drug screening. Although the pathogenesis of cholestatic liver diseases like PSC remains unknown, the importance of the vascular-biliary interface is clear. Cholangiocyte injury not only impairs barrier function such that bile leaks and damages periductal tissue, but also...

Autoimmune hepatitis (AIH) is a chronic inflammatory disease that results from autoantibody mediated hepatocyte injury. Given its immune-mediated mechanism, it is more likely to present with other autoimmune conditions, particularly involving thyroid gland. In patients presenting with both Graves' disease and hepatitis, the diagnostic challenge is to determine the cause of elevated liver enzymes. We present an 11-year-old girl with acute liver cell failure and cholestasis in a setting of...

Effective therapies for primary sclerosing cholangitis (PSC), a progressive cholestatic liver disease characterized by biliary inflammation and fibrotic damage, remain limited due to an incomplete elucidation of its underlying molecular mechanisms. Although N6-methyladenosine (m6A) RNA methylation has been implicated in hepatic pathophysiology, its role in PSC remains undefined. Here, we demonstrate that hepatocyte-specific deletion of Mettl3, a critical m6A methyltransferase, induces...

CONCLUSIONS: These findings suggest the potential of the biliary microbiome as a diagnostic tool, offering insights into the pathophysiology and possible therapeutic targets for HPB diseases. However, given the heterogeneity in methodologies and the limited number of studies including healthy controls, these observations remain preliminary; further prospective validation is required before clinical translation.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with no effective curative therapies. Necroptosis, a regulated necrotic cell death pathway controlled by receptor-interacting protein kinase 1 (RIPK1), has emerged as a potential driver of inflammation and fibrosis in chronic liver disorders. We investigated the role of necroptosis in PSC and whether RIPK1 inhibition could modify disease course and progression. Spatial profiling of human PSC biopsies revealed that...

CONCLUSION: Primary sclerosing cholangitis patients undergoing ERCP have high symptom burden and rates of neoplastic pathology, with high rates of recurrent obstruction post-ERCP. Stent insertion is associated with a sicker PSC phenotype and higher risk of post-ERCP complications. Future studies are needed to further explore the role of ERCP in management of PSC.

CONCLUSIONS: HRU and healthcare costs for patients with PSC in Sweden were substantial and significantly increased after diagnosis. Effective therapies are needed to reduce disease progression and economic burden.

CONCLUSIONS: While having comparable extracellular effects on collagen crosslinking in vivo, the cell-permeable LOXL2 inhibitor PAT-1251 exerted potent antifibrotic activity in hepatic progenitors and HSC cultures compared with the anti-LOXL2 antibody. PAT-1251 substantially outperformed the anti-LOXL2 antibody in the BALB/c. Mdr2-/- model of biliary fibrosis, suggesting that intracellular LOXL2 targeting is therapeutically important in addition to its well-characterized extracellular collagen...

CONCLUSION: Our study highlights the burden of pruritus experienced by participants across CLDs, highlighting a need to improve symptom recognition and treatments focused on improving HRQoL.

Background/Objectives: Biliary reconstruction in liver transplantation (LT) for primary sclerosing cholangitis (PSC) is controversial. A Roux-en-Y hepaticojejunostomy (HJ) is associated with fewer anastomotic strictures, while a duct-to-duct reconstruction (DD) shows a decreased rate of cholangitis and preserves anatomy for endoscopy. The aim of our study was to analyze patient survival and postoperative outcomes after LT for PSC based on the type of reconstruction in two high-volume LT centers....

Background: Primary Sclerosing Cholangitis (PSC), Primary Biliary Cholangitis (PBC), and Autoimmune Hepatitis (AIH) are rare immune-mediated liver conditions that significantly affect patients' quality of life. In Romania, access to specialized information and patient support resources is limited, underscoring the need for tailored educational tools. The aim was to describe the methodology for developing, implementing, and conducting a feasibility study of an online platform for patients with...

Immune checkpoint inhibitors (ICIs) are increasingly being used as part of cancer treatment. Whilst effective oncological therapies, these drugs are associated with a significant risk of immune-related adverse events (IrAEs). They can affect a variety of organ systems, including the lungs, heart, kidneys, and gastrointestinal tract, with liver damage being a relatively well-recognised complication. There have been multiple previous reports describing immune-related hepatitis; however, more...

Triple overlap syndrome involving autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) is an exceptionally rare autoimmune hepatopathy, posing major diagnostic and therapeutic challenges. A 51-year-old woman initially diagnosed with AIH-PBC overlap presented persistent hepatocellular cytolysis despite corticosteroids and ursodeoxycholic acid. Autoimmune screening revealed ANA, anti-M2, and anti-gp210 positivity. Repeat liver biopsy showed...

CONCLUSION: This study found a link between miR-21-5p dysregulation and gut microbiota dysbiosis, colonic inflammation, and pro-oncogenic signaling in PSC-IBD patients. These findings highlight miR-21-5p as a potential modulator of disease progression and neoplastic risk.

CONCLUSION: One in 4 PSC patients experience moderate-severe pruritus, with greater symptom intensity in those with advanced disease. Our dataset is able to serve as a reference tool to aid future interventional study design, with regards anti-pruritus therapies in PSC.

CONCLUSIONS: PSC is less prevalent among Asian patients with IBD than in Western populations. The increased use of magnetic resonance cholangiopancreatography may enable earlier detection, contributing to milder disease severity and improved clinical outcomes in recent years.

CONCLUSION: IBD-PSC is associated with substantially elevated risks of both intestinal and extraintestinal malignancies compared to isolated IBD or PSC. These findings highlight the synergistic oncogenic potential of coexisting IBD and PSC and underscore the need for tailored surveillance and early detection strategies in this high-risk population.

CONCLUSIONS: AILD represents a growing clinical challenge in the Asia-Pacific region. Earlier recognition, individualized treatment strategies, and strengthened multinational collaboration are essential to address unmet diagnostic and therapeutic needs and to improve long-term outcomes.

CONCLUSIONS: While favorable survival in confirmed dysplasia supports the role of preemptive LT, the absence of neoplasia in a substantial proportion of explants, particularly in suspected LGD, calls for a cautious, individualized approach. LT appears more clearly justified in accurately diagnosed HGD, given its strong association with early malignancy and the poor prognosis of advanced CCA.

Background and Aims: Metabolic dysfunction-associated steatotic liver disease (MASLD) is the most common cause of liver disease in the United States and frequently coexists with other liver diseases. Despite growing interest, the presence of MASLD in patients with primary sclerosing cholangitis (PSC) remains underexplored. This study aimed to assess the prevalence and characteristics of the MASLD-PSC overlap syndrome, with a specific focus on patient-reported outcomes such as pruritus and...

CONCLUSIONS: Profound alterations in serum and tissue levels of primary and taurine-conjugated BAs occur during cholangiocarcinogenesis. Further elucidation of the mechanisms regulating BA and oxysterol metabolism in CCA may offer new avenues for diagnosis and therapy.