Background: The cornerstone treatment for primary biliary cholangitis (PBC) is ursodeoxycholic acid (UDCA), but many patients exhibit an incomplete response, leading to disease progression. Risk prediction models like the GLOBE and UK-PBC scores hold promise for patient stratification and management. We aimed to independently assess the predictive accuracy of these risk scores for UDCA response in a prospective U.S. cohort. Methods: We conducted a prospective cohort study at a U.S. liver center, monitoring UDCA-treated PBC patients over a one-year follow-up. We evaluated the predictive efficacy of the GLOBE and UK-PBC scores for UDCA treatment response, comparing them to the Paris II criteria. Efficacy was assessed using univariate and multivariate analyses, followed by prognostic performance evaluation via receiver operating characteristic (ROC) curve analysis. Results: We evaluated 136 PBC patients undergoing UDCA therapy. Based on the Paris II criteria, patients were categorized into UDCA full-response and non-response groups. The GLOBE score identified a non-responder rate of 18% (p = 0.205), compared to 20% (p = 0.014) with the Paris II criteria. Multivariate analysis, adjusted for age and biochemical markers, showed that both the GLOBE and UK-PBC scores were strongly associated with treatment response (p < 0.001). The area under the ROC curve was 0.87 (95% CI 0.83−0.95) for the GLOBE score and 0.94 (95% CI 0.86−0.99) for the UK-PBC risk score. Conclusions: Our study demonstrates that GLOBE and UK-PBC scores effectively predict UDCA treatment response in PBC patients. The early identification of patients at risk of an incomplete response could improve treatment strategies and identify patients who may need second-line therapies.

Background: Acute liver failure (ALF) involves rapid liver injury, often leading to multi-organ failure. Liver transplantation (LT) has improved survival rates, with U.S. rates reaching 92%. This study analyzes UNOS data (2002–2020) to evaluate long-term survival and identify risk factors affecting waitlist and post-LT outcomes in ALF patients. Methods: A retrospective analysis was performed on adult ALF patients waitlisted for LT (Status 1/1A). ALF etiologies, including viral infections, drug-induced liver injury (DILI), acetaminophen (APAP) overdose, autoimmune hepatitis (AIH), Wilson disease (WD), and unknown causes, were assessed with patient and donor characteristics. Kaplan–Meier and Cox regression analyses identified predictors of patient and graft survival. Sensitivity analysis confirmed the model’s robustness. Results: We identified 2759 ALF patients. APAP (HR 1.7; p < 0.001) and unknown etiology (HR 1.3; p = 0.037) were linked to higher waitlist removal risk, while WD (HR 0.36; p < 0.001) increased LT probability. Among 2014 LT recipients, WD showed improved survival (HR 0.53; p = 0.002). Black/African American race (HR 1.47; p < 0.001), diabetes (HR 1.81; p < 0.001), and encephalopathy (HR 1.27; p < 0.001) predicted higher mortality. AIH had the lowest 1- and 10-year survival (83% and 62%), while APAP had the lowest 5-year survival (76%). WD had the highest graft survival at 1, 5, and 10 years (93%, 88%, and 80%). Conclusions: ALF etiology significantly affects survival outcomes. AIH and APAP are associated with worse survival, while WD shows favorable outcomes. Tailored post-LT management is essential to improve survival in ALF patients.

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. Methods: We analyzed data from 109 adults with PSC enrolled in a prospective registry (2018–2024) with ≥4 years of follow-up. Logistic regression identified the predictors of LT or death, and the model performance was assessed using the area under the receiver operating characteristic curve (AUROC). Multicollinearity was evaluated using the variance inflation factor (VIF). Results: Among the 109 patients (mean age 45 ± 15 years, 51% female), 85% remained alive without LT, 12% underwent LT, and 3% died over a median follow-up of 4.63 years. The MRS was significantly associated with LT or death (OR 3.08, p < 0.001) and demonstrated excellent predictive performance (AUROC 0.99, p < 0.001). The model achieved 95.45% sensitivity, 98.85% specificity, and a correct classification rate of 98.17%, supporting its clinical utility. Conclusion: The MRS is a robust tool for risk stratification in PSC, predicting LT and mortality. These findings highlight its broader applicability beyond end-stage PSC and underscore its potential for guiding clinical management and early intervention strategies.

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Autoimmune hepatitis (AIH) is a varied inflammatory chronic liver disease. AIH’s prevalence varies and has increased recently. Diagnosis involves the discovery of histologic features following liver biopsy and serologic testing. Clinical features vary, and up to 40% of patients may be asymptomatic. Evaluating thiopurine methyltransferase (TMPM) activity before treatment is crucial for an optimal response. The primary treatment goal is biochemical remission, normalized serum IgG, and liver enzymes. Induction therapy typically involves azathioprine and corticosteroids. Close monitoring of liver function tests and serum immunoglobulin levels is essential. Medications can be tapered after achieving biochemical remission. Liver transplantation may be required for refractory disease or cirrhosis. Further therapeutic approaches are needed, particularly for non-responders to first-line treatments.

Primary biliary cholangitis (PBC) prompts liver transplantation (LT) due to cholestasis, cirrhosis, and liver failure. Despite lower MELD scores, recent studies highlight higher PBC waitlist mortality, intensifying the need for alternative transplantation strategies. Living donor liver transplant (LDLT) has emerged as a solution to the organ shortage. This study compares LDLT and deceased donor liver transplant (DDLT) outcomes in PBC patients via retrospective analysis of the UNOS database (2002–2021). Patient survival, graft failure, and predictors were evaluated through Kaplan–Meier and Cox-proportional analyses. Among 3482 DDLTs and 468 LDLTs, LDLT showed superior patient survival (92.3%, 89.1%, 87.6%, 85.0%, 77.2% vs. 91.5%, 88.3%, 86.3%, 82.2%, 71.0%; respectively; p = 0.02) with no significant graft survival difference at 1-, 2-, 3-, 5-, and 10-years post-LT (91.0%, 88.0%, 85.7%, 83.0%, 75.4% vs. 90.5%, 87.4%, 85.3%, 81.3%, 70.0%; respectively; p = 0.06). Compared to DCD, LDLT showed superior patient and graft survival (p < 0.05). Younger male PBC recipients with a high BMI, diabetes, and dialysis history were associated with mortality and graft failure (p < 0.05). Our study showed that LDLT had superior patient survival to DDLT. Predictors of poor post-LT outcomes require further validation studies.