Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are autoimmune liver diseases with strong female predominance. They are caused by T cell-mediated injury of hepatic parenchymal cells, but the mechanisms underlying this sex bias are unknown. Here, we investigated whether testosterone contributes to T cell activation in women with PBC. Compared with sex- and age-matched healthy controls (n = 23), cisgender (cis) women with PBC (n = 24) demonstrated decreased testosterone serum...

Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is a distinct form of sclerosing cholangitis frequently associated with autoimmune pancreatitis and is recognized as a biliary manifestation of IgG4-related disease. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasonography (EUS) are key diagnostic modalities for IgG4-SC. Cholangiocarcinoma and primary sclerosing cholangitis (PSC) are significant mimickers of IgG4-SC. ERCP is employed to evaluate...

CONCLUSION: The precision of quantitative MRCP-derived metrics were sufficient to differentiate PSC and healthy subjects and should be well suited for multi-centre trials and assessment of biliary tree health.

CONCLUSION: Hepatocellular carcinoma is relatively rare in patients with primary sclerosing cholangitis who do not have cirrhosis, especially in those under the age of 50. Our findings indicate that HCC monitoring for patients with PSC can be tailored, based on their age and cirrhosis status.

CONCLUSIONS: The SCOPE index effectively predicts LT in pediatric PSC, with strong reliability over time. Coexisting AIH may affect score accuracy at diagnosis due to inflammation.

Inflammatory bowel disease (IBD) in the pediatric population presents distinct characteristics compared to adult cases. Pathology plays a critical role in its diagnosis, and this review underscores key considerations in the pathologic evaluation of pediatric IBD. Recognizing inflammatory patterns in the upper gastrointestinal tract can improve disease classification and aid in diagnosing IBD in certain scenarios, such as isolated upper gastrointestinal or small bowel involvement. Additionally,...

CONCLUSIONS: The recurrence rate was high in pediatric patients with PSC. The observed association with immune-activating conditions raises the possibility of utilizing immunologic interventions to prevent rPSC, although further prospective studies are warranted to clarify the underlying mechanisms.

Explore the causal relationship of risk between type 1 diabetes and primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). A causal association between type 1 diabetes and autoimmune liver disease remains ambiguous. This study explored potential causality between different autoimmune conditions, indicating that caution should be taken of the occurrence of autoimmune liver diseases in daily management of T1D patients. Genetic variants were extracted as instrumental variables...

CONCLUSION: The study illustrates the potential of examining CT imaging using 3D-DenseNet121 deep learning model to predict hepatic decompensation in patients with PSC.

The three major autoimmune liver diseases are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).These conditions are assumed to result from a breakdown in immunological tolerance, which leads to an inflammatory process that causes liver damage.The self-attack is started by T-helper cell-mediated identification of liver autoantigens and B-cell production of autoantibodies,and it is maintained by a reduction in the number and activity of...

Recent findings have suggested that gallbladder-derived retinoic acid signaling plays a crucial role in the regeneration of damaged intrahepatic biliary ducts. This retrospective cohort study analyzed the clinical records of 20 patients with primary sclerosing cholangitis (PSC) treated at our hospital between 2013 and 2024. We investigated the clinical implications of gallbladder removal in patients with PSC, a progressive cholangiopathy with limited therapeutic options. We retrospectively...

RATIONALE: Primary sclerosing cholangitis (PSC) is characterized by idiopathic intra- and extrahepatic bile duct inflammation and biliary fibrotic changes. Recurrent bile duct stones due to PLKR and UGT1A1 mutation is an extremely rare complications of PSC.

CONCLUSIONS: PSC significantly increases the risk of short-term complications in hospitalized IBD patients and the likelihood of chronic liver disease-related complications. These findings highlight the need for targeted management strategies for IBD patients with co-existing PSC.

Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are rare liver diseases that significantly impair quality of life (QoL). In this study, we analysed two large cohorts comprising a total of 1267 patients with PBC and PSC, showing that fatigue is a frequent symptom in both conditions, particularly among females. Fatigue was associated with liver function markers (ALP/GGT in PSC) and with cirrhosis (in PBC). It was also often linked to cholestatic pruritus, further...

CONCLUSION: MR analysis confirmed a causal association between anti- H. pylori IgG and UC, but not with CD. The genus.Anaerofilum may increase the risk of UC by inhibiting H. pylori infection.

The main cholestatic liver diseases comprise primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), and IgG4-related cholangitis (IgG4-C). Despite being classified as rare diseases these are becoming gradually more important in the field of hepatology since their incidence is slowly rising while viral hepatitis burden is declining. Cholestatic liver diseases now rank among the three most frequent indications for liver transplantation in many Western countries. An accurate...

CONCLUSIONS: Our RSF model offers more accurate waitlist outcome prediction in PSC. The significant performance improvement with the inclusion of PSC-specific variables highlights the importance of disease-specific variables for predicting trajectories of clinically distinct presentations.

CONCLUSION: This study indicates a potential association between circulating inflammatory proteins and AILD, which may become a new diagnostic indicator or drug target for clinical application in the prevention and treatment of AILD. However, further investigation is needed.

Background: The Mayo Risk Score (MRS) predicts short-term mortality in primary sclerosing cholangitis (PSC) using the age, bilirubin, albumin, aspartate aminotransferase (AST), and variceal bleeding history. While the MRS has been validated in end-stage PSC, its ability to predict liver transplantation (LT) and outcomes in newly diagnosed patients without advanced disease remains unclear. This study evaluated the effectiveness of the MRS in predicting LT and mortality in this patient population....

Primary sclerosing cholangitis (PSC) is a progressive auto-inflammatory condition of the biliary ducts clinically characterized by painless cholestasis and jaundice. Histologically, the typical findings in PSC are periductal fibrosis with inflammation, bile duct proliferation, and ductopenia. These hallmarks eventually develop into end-stage liver disease requiring liver transplantation (LT), although the latency between diagnosis and LT is variable among patients. PSC is the leading indication...

GPR35 is an orphan G-protein coupled receptor that has been implicated in the development of cancer. GPR35 regulates the Na^(+)/K^(+)-ATPase's pump and signalling function. Here we show GPR35's critical role in ion flux that in turn controls cellular osmotic pressure and Na^(+)-dependent transport in HepG2 and SW480 cells. GPR35 deficiency results in increased levels of intracellular Na^(+), osmotic stress and changes in osmolytes leading to increased cells size and decreased glutamine import in...

Multiple sclerosis (MS) is a chronic autoimmune condition characterized by central nervous system demyelination. Its coexistence with primary biliary cholangitis (PBC), another autoimmune disease, is rarely described in the literature. This is the case of a 35-year-old Emirati female patient with a history of PBC who presented with unsteady gait and neurological symptoms. Neurological examination revealed dysmetria, wide-based gait, and brisk reflexes. MRI findings confirmed MS, and she was...

CONCLUSION: The 3D DenseNet-121 MRI model effectively detects early-stage pCCA in PSC patients. Compared to expert radiologists, the model missed fewer cases of cancer.

CONCLUSIONS: We have developed a model framework to simulate the progression of PSC with estimates of overall and transplant-free survival. This model, which calibrates well with existing estimates of disease progression, may be useful to evaluate the clinical and economic benefits of future treatments.

Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation and progressive fibrosis of the biliary tree, leading to significant liver function impairment over time. There is a strong association with inflammatory bowel diseases (IBD), together representing a distinct and complex medical condition. Patients with PSC-IBD face a heightened risk of various cancers, particularly colorectal carcinoma (CRC) and cholangiocarcinoma (CCA) as the most...

Autoimmune liver diseases (AiLDs), including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC), are immune-mediated conditions associated with significant hepatic and systemic manifestations. Among these, cytopenias-defined as reductions in blood cell counts affecting single or multiple lineages-represent a clinically important, though often under-recognized, complication. Cytopenias in AiLDs arise from diverse mechanisms, including...

CONCLUSIONS: Our research pioneered the identification of CDC42 as a target for slowing PSC progression. Our research not only uncovers a possible drug target but also provides direction for the development of therapeutics for PSC.

CONCLUSION: Our data highlight the association of E. faecalis and faecal cytolysin with lower survival in patients with PSC. These data should prompt further research into the pathogenic role of cytolysin-positive E. faecalis, and to explore its role as a potential therapeutic target.

Primary sclerosing cholangitis (PSC) is a chronic biliary inflammation associated with periductular fibrosis of the intrahepatic and extrahepatic bile ducts leading to strictures, bacterial cholangitis, decompensated liver disease and need for liver transplantation. This rare focal liver disease affects all races and ages, with a predominance of young males. There is an up to 88% association with inflammatory bowel disease. Although the aetiology is unknown and the pathophysiology is poorly...

CONCLUSIONS: Black patients had sociodemographic and clinical features suggestive of more severe disease compared to White patients. Although hospitalization rates were similar, Black patients received higher-intensity care. There was no significant difference in liver transplantation between Black and White patients. Additional research on optimal diagnosis and management of PSC in Black populations specifically is necessary to reduce healthcare disparities in this disease.