Current Perspectives on the Molecular and Clinical Relationships between Primary Biliary Cholangitis and Hepatocellular Carcinoma

Floreani, Annarosa; Gabbia, Daniela; De Martin, Sara

doi:10.3390/ijms25042194

Cited by 5 publications

(3 citation statements)

References 124 publications

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“…Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by immune-mediated destruction of small and medium intrahepatic bile ducts that lead to cholestasis [54] and eventually progresses to cirrhosis and liver failure [55,56], characterized by a complex and not completely defined pathogenetic mechanism [57]. The incidence and prevalence of PBC differ according to region, with increased prevalence in Northern Europe [58].…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

Are Gender Differences Important for Autoimmune Liver Diseases?

Floreani,

Gabbia,

De Martin

2024

Life

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Gender Medicine has had an enormous expansion over the last ten years. Autoimmune liver diseases include several conditions, i.e., autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and conditions involving the liver or biliary tree overlapping with AIH, as well as IgG4-related disease. However, little is known about the impact of sex in the pathogenesis and natural history of these conditions. The purpose of this review is to provide an update of the gender disparities among the autoimmune liver diseases by reviewing the data published from 1999 to 2023. The epidemiology of these diseases has been changing over the last years, due to the amelioration of knowledge in their diagnosis, pathogenesis, and treatment. The clinical data collected so far support the existence of sex differences in the natural history of autoimmune liver diseases. Notably, their history could be longer than that which is now known, with problems being initiated even at a pediatric age. Moreover, gender disparity has been observed during the onset of complications related to end-stage liver disease, including cancer incidence. However, there is still an important debate among researchers about the impact of sex and the pathogenesis of these conditions. With this review, we would like to emphasize the urgency of basic science and clinical research to increase our understanding of the sex differences in autoimmune liver diseases.

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Section: Primary Biliary Cholangitismentioning

confidence: 99%

Are Gender Differences Important for Autoimmune Liver Diseases?

Floreani,

Gabbia,

De Martin

2024

Life

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“…Primary biliary cholangitis (PBC) is a chronic cholestatic immune liver disease characterized by persistent cholestasis, interlobular bile duct damage, liver fibrosis, eventual cirrhosis, and death [ 1 ]. Its prevalence in Europe has an overall rate of 22.27 cases per 100,000 inhabitants and a pooled incidence rate of 1.7 new cases per 100,000 inhabitants per year, with a female predominance (1.6–4.8:1 female: male ratio) [ 2 ]. There are still approximately 30% of patients with PBC who are not responsive to ursodesoxycholic acid (UDCA), which is the only drug currently approved by the FDA for application in cholestatic liver disease [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Distinct signatures of gut microbiota and metabolites in primary biliary cholangitis with poor biochemical response after ursodeoxycholic acid treatment

Han,

Song,

Huang

et al. 2024

Cell Biosci

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Background About 1/3 of primary biliary cholangitis (PBC) patients suffered from poor response worldwide. And these patients present intestinal disturbances. We aimed to identify signatures of microbiota and metabolites in PBC patients with poor response, comparing to patients with response. Methods This study enrolled 25 subjects (14 PBC patients with response and 11 PBC patients with poor response). Metatranscriptomics and metabolomics analysis were carried out on their fecal. Results PBC patients with poor response had significant differences in the composition of bacteria, characterized by decreased Gemmiger etc. and increased Ruminococcus etc. The differential microbiota functions characterized by decreased abundance of elongation factor Tu and elongation factor G base on the KO database, as well as decreased abundance of Replicase large subunit etc. based on the SWISS-PROT database. PBC with poor response also had significant differences in 17 kinds of bacterial metabolites, characterized by decreased level of metabolites vital in bile acids metabolism pathway (L-Cysteine etc.) and the all-trans-Retinoic acid, a kind of immune related metabolite. The altered microbiota was associated with the differential expressed metabolites and clinical liver function indicators. 1 bacterial genera, 2 bacterial species and 9 metabolites simultaneously discriminated PBC with poor response from PBC with response with high accuracy. Conclusion PBC patients with poor response exhibit unique changes in microbiota and metabolite. Gut microbiota and metabolite-based algorithms could be used as additional tools for differential prediction of PBC with poor prognosis.

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“…Another disease that may increase the risk for HCC development is primary biliary cholangitis (PBC), a chronic autoimmune liver disease characterized by the immune-mediated destruction of bile ducts. Floreani and collaborators summarize the state of the art regarding the molecular alterations shared by PBC and HCC and the risk factors that may be predictive for HCC in PBC patients [10].…”

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confidence: 99%