Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: a Review Featuring a Women's Health Perspective

Beery, Renée M. Marchioni; Vaziri, Haleh; Forouhar, Faripour

doi:10.14218/jcth.2014.00024

Cited by 19 publications

(25 citation statements)

References 197 publications

(347 reference statements)

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“…Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are rare, chronic idiopathic liver diseases characterized by immune damage and blockage of bile ducts in the liver. While small intrahepatic bile ducts are affected in PBC, medium and large-sized extrahepatic and/or intrahepatic bile ducts are impaired in PSC [ 1 ]. The etiology of either diseases is unknown although several contributing factors have been reported.…”

mentioning

confidence: 99%

Genome-wide Resolution Peripheral Blood Methylome Profiling Reveals Signatures for Cholestatic Liver Disease

et al. 2020

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Aim: To profile DNA methylation changes of primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Materials & methods: Patients with: PBC, PSC with inflammatory bowel disease (IBD), PSC without IBD, and age-, sex-matched controls were profiled for methylomes of peripheral blood by reduced representation bisulfite sequencing. Differentially methylated CpG (DMC) and differentially methylated region (DMR) were detected and compared. Results: We identified consistently altered DMCs and DMRs across diseases with involvement in key pathways. Many similarities noted between two subtypes of PSC, interestingly few existed between PBC and PSC. DMRs were highly enriched with transcription factor binding. Top DMC changes were validated in liver tissue of an independent cohort. Conclusion: Methylome profiling provides insights to PBC and PSC.

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mentioning

confidence: 99%

Genome-wide Resolution Peripheral Blood Methylome Profiling Reveals Signatures for Cholestatic Liver Disease

et al. 2020

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“…The clinical course of PSC is highly variable, with a median survival rate of 12-18 years from diagnosis or until liver transplantation; whereas, the median survival rate for PBC has been noted to be much shorter, 9.3 years. 2,6 Due to the small sample size of PSC-PBC overlap cases, though, the data cannot be accurately extrapolated to estimate the morbidity and mortality statistics for patients thought to have overlap disease.…”

Section: Epidemiologymentioning

confidence: 99%

“…Both PSC and PBC are slow progressive diseases which occur over decades. 6 The pathogenesis of both PBC and PSC is incompletely understood.…”

Section: Etiology/proposed Mechanismsmentioning

confidence: 99%

“…Less commonly, cases had radiological evidence supporting PSC and PBC co-existence at the time of initial diagnosis. 6,23 Clinical features and symptomatology Typical symptoms common to both intrahepatic PSC and PBC include fatigue, pruritis, and jaundice, all of which are due to the cholestatic process. 6 PSC patients may also experience right upper abdominal quadrant pain, fevers and chills, if the cholestasis causes obstruction or infection or bacterial cholangitis.…”

Section: Etiology/proposed Mechanismsmentioning

confidence: 99%

“…Less commonly, cases had radiological evidence supporting PSC and PBC co-existence at the time of initial diagnosis. 6 , 23 …”

Section: Etiology/proposed Mechanismsmentioning

confidence: 99%

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Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Overlap Syndrome: A Review

Mago¹,

Wu²

2020

Journal of Clinical and Translational Hepatology

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Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are slow progressive diseases which have been increasing in prevalence. The pathogeneses of PBC and PSC are incompletely understood but the underlying mechanisms appear to be fundamentally autoimmune in origin. Although PBC and PSC appear to be separate entities, overlap has been described. Diagnosis depends on a combination of serological markers, imaging, and pathological criteria. The mainstay of treatment has been ursodeoxycholic acid and in some cases of extrahepatic biliary obstruction and overlap disorder, endoscopic retrograde cholangiopancreatography has been useful.

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H19 promotes cholestatic liver fibrosis by preventing ZEB1‐mediated inhibition of epithelial cell adhesion molecule

et al. 2017

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Based on our recent finding that disruption of bile acid (BA) homeostasis in mice results in the induction of hepatic lncRNA H19 expression, we sought to elucidate the role of H19 in cholestatic liver fibrosis. Hepatic overexpression of H19RNA augmented bile duct ligation (BDL)-induced liver fibrosis, which was accompanied by the elevation of serum ALT, AST, bilirubin, and BA levels. Multiple genes related to liver fibrosis, inflammation, and biliary hyperplasia were increased in H19-BDL vs Null-BDL mice, whereas genes in BA synthesis were decreased. Livers and spleens of H19-BDL mice showed significant enrichment of CD3+γδ+, IL-4, and IL-17 producing CD4+ and CD8+ immune cell populations. H19 downregulated hepatic zinc finger E-box-binding homeobox 1 (ZEB1) but upregulated epithelial cell adhesion molecule (EpCAM) and SRY (sex determining region Y)-box 9 (SOX9) expression. Mechanistically, ZEB1 repressed EpCAM promoter activity and gene transcription. H19RNA impeded ZEB1’s inhibitory action by interacting with ZEB1 protein to prevent its binding to the EpCAM promoter. Hepatic overexpression of ZEB1 or knockdown of EpCAM diminished H19-induced fibrosis; the latter was also prevented in H19−/− mice. H19RNA was markedly induced by bile acids in mouse small cholangiocytes (MSC) and to a lesser extent in mouse large cholangiocytes (MLC). The upregulation of H19RNA and EpCAM correlated positively with the downregulation of ZEB1 in primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) liver specimens. Conclusions: The activation of hepatic H19RNA promoted cholestatic liver fibrosis in mice through the ZEB1/EpCAM signaling pathway.

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Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: a Review Featuring a Women's Health Perspective

Cited by 19 publications

References 197 publications

Genome-wide Resolution Peripheral Blood Methylome Profiling Reveals Signatures for Cholestatic Liver Disease

Genome-wide Resolution Peripheral Blood Methylome Profiling Reveals Signatures for Cholestatic Liver Disease

Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Overlap Syndrome: A Review

H19 promotes cholestatic liver fibrosis by preventing ZEB1‐mediated inhibition of epithelial cell adhesion molecule

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