Primary biliary cholangitis is an autoimmune cholestatic liver disease characterized by progressive destruction of bile ducts, which can ultimately progress to chronic liver disease and cirrhosis. Ursodeoxycholic acid and obeticholic acid are the only 2 Food and Drug Administration (FDA)-approved medications for primary biliary cholangitis. Unfortunately, up to 40% of patients with primary biliary cholangitis have an incomplete response to ursodeoxycholic acid, warranting an essential need for additional therapeutics. Peroxisome proliferator-activated receptor agonists have shown promising data supporting their use as disease-modifying therapies. Fibroblast growth factor-19 agonists, farnesoid X receptor agonists, and nicotinamide adenine dinucleotide phosphate (NADPH) oxidase 3 inhibitors are additional agents under investigation as potential disease-modifying therapy. However, evidence supporting the use of certain novel therapies over others is sparse. There is a need for additional clinical trials as well as research aimed at the underlying pathophysiology of primary biliary cholangitis to discover additional therapeutic targets. Cite this article as: Barba Bernal R, Ferrigno B, Medina Morales E, et al. Management of primary biliary cholangitis: Current treatment and future perspectives. Turk J Gastroenterol. 2023;34(2):89-100.

Primary sclerosing cholangitis (PSC) is the leading indication of liver transplantation (LT) among autoimmune liver disease patients. There is a scarcity of studies comparing survival outcomes between living-donor liver transplants (LDLT)s and deceased-donor liver transplants (DDLTs) in this population. Using the United Network for Organ Sharing database, we compared 4679 DDLTs and 805 LDLTs. Our outcome of interest was post-LT patient survival and post-LT graft survival. A stepwise multivariate analysis was performed, adjusting for recipient age, gender, diabetes mellitus, ascites, hepatic encephalopathy, cholangiocarcinoma, hepatocellular carcinoma, race, and the model for end-stage liver disease (MELD) score; donor’ age and sex were also included to the analysis. According to univariate and multivariate analysis, LDLT had a patient and graft survival benefit compared to DDLT (HR, 0.77, 95% CI 0.65–0.92; p < 0.002). LDLT patient survival (95.2%, 92.6%, 90.1%, and 81.9%) and graft survival (94.1%, 91.1%, 88.5%, and 80.5%) at 1, 3, 5, and 10 years were significantly better than DDLT patient survival (93.2%, 87.6%, 83.3%, and 72.7%) and graft survival (92.1%, 86.5%, 82.1%, and 70.9%) (p < 0.001) in the same interval. Variables including donor and recipient age, male recipient gender, MELD score, diabetes mellitus, hepatocellular carcinoma, and cholangiocarcinoma were associated with mortality and graft failure in PSC patients. Interestingly, Asians were more protected than Whites (HR, 0.61; 95% CI, 0.35–0.99; p < 0.047), and cholangiocarcinoma was associated with the highest hazard of mortality (HR, 2.07; 95% CI, 1.71–2.50; p < 0.001) in multivariate analysis. LDLT in PSC patients were associated with greater post-transplant patient and graft survival compared to DDLT patients.

Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are two types of chronic cholestatic liver disease (CCLD). Little is known regarding the relationship between these conditions and pregnancy. We performed a systematic review and meta-analysis regarding the maternal and fetal outcomes amongst patients with a known diagnosis of PBC and PSC undergoing pregnancy. Our analysis shows that patients with PBC and PSC who undergo pregnancy are at an increased risk of pre-term delivery, as well as the development of new or worsening pruritus during pregnancy. Additionally, patients with PBC are at higher risk of undergoing a biochemical disease flare during the postpartum period compared to during pregnancy. However, there were no documented cases of maternal mortality or development of decompensated cirrhosis during pregnancy or the postpartum period.

An intramural duodenal hematoma (IDH) is an uncommon clinical entity that can be an adverse event of therapeutic esophagogastroduodenoscopy (EGD). Herein, we present a case of a 60-year-old man who presented to the hospital with vomiting, diarrhea, and overt gastrointestinal bleeding with melena and small-volume hematemesis. EGD was pursued and revealed a bleeding duodenal ulcer that was therapeutically intervened upon to achieve hemostasis. After endoscopy, his hospital course was complicated by an intramural duodenal hematoma, which presented with abdominal pain and worsening anemia without recurrent overt gastrointestinal bleeding. After consultation with interventional radiology and surgery, he ultimately improved with supportive care and was discharged home without further endoscopic or procedural intervention. An IDH is an uncommon gastrointestinal pathology that should be considered after endoscopy in the setting of abdominal pain and anemia without overt bleeding. Conservative management often leads to successful outcomes, but multidisciplinary care should be considered in the setting of further adverse events.