Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management

Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that...

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Veröffentlicht in:	Gastroenterology (New York, N.Y. 1943) Jg. 145; H. 3; S. 521
Hauptverfasser:	Eaton, John E, Talwalkar, Jayant A, Lazaridis, Konstantinos N, Gores, Gregory J, Lindor, Keith D
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	United States 01.09.2013
Schlagworte:	Bile Duct Neoplasms - diagnosis Bile Duct Neoplasms - etiology Bile Duct Neoplasms - therapy Bile Ducts, Intrahepatic Cholagogues and Choleretics - therapeutic use Cholangiocarcinoma - diagnosis Cholangiocarcinoma - etiology Cholangiocarcinoma - therapy Cholangiopancreatography, Endoscopic Retrograde Cholangitis, Sclerosing - diagnosis Cholangitis, Sclerosing - etiology Cholangitis, Sclerosing - therapy Colonic Neoplasms - diagnosis Colonic Neoplasms - etiology Colonic Neoplasms - therapy Combined Modality Therapy Diagnosis, Differential Gallbladder Neoplasms - diagnosis Gallbladder Neoplasms - etiology Gallbladder Neoplasms - therapy Genetic Predisposition to Disease Humans Immunity, Innate Immunosuppressive Agents Intestines - microbiology Liver Transplantation Metagenome Ursodeoxycholic Acid - therapeutic use magnetic resonance LT UDCA TNF UC endoscopic retrograde cholangiopancreatography Autoimmune Disease gallbladder neoplasia Th MDR ulcerative colitis Inflammatory Bowel Disease carbohydrate antigen Toll-like receptor hepatocellular carcinoma AIH PSC IL MR janus kinase mucosal addressin cellular adhesion molecule 1 AIP LGD HCC autoimmune pancreatitis IAC fluorescence in situ hybridization VAP-1 CFTR CRN Ig LPC primary sclerosing cholangitis FXR Cholestatic Liver Disease immunoglobulin G4–associated cholangitis IBD low-grade dysplasia GBN T-helper autoimmune hepatitis ursodeoxycholic acid lymphoplasmacytic TLR interleukin liver transplant CA Cholangiocarcinoma CCA MAdCAM-1 colorectal neoplasia immunoglobulin farnesoid X receptor vascular adhesion protein 1 multidrug resistance protein tumor necrosis factor JAK MRCP FISH cystic fibrosis transmembrane conductance regulator ERCP magnetic resonance cholangiopancreatography
ISSN:	1528-0012, 1528-0012
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Beschreibung
Zusammenfassung:	Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood, which in part explains the lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies.
Bibliographie:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 ObjectType-Review-3 content type line 23 ObjectType-Undefined-4
ISSN:	1528-0012 1528-0012
DOI:	10.1053/j.gastro.2013.06.052