Cholestatic Liver Disease: Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Drug-Induced Cholestasis Keith D. Lindor, M.D

2005 ◽  
pp. 345-348
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Drug Induced

scholarly journals Medical Management of Chronic Cholestatic Liver Diseases

2000 ◽  
Vol 14 (suppl d) ◽  
pp. 93D-98D ◽  
Author(s):  
Andrea A Gossard ◽  
Keith D Lindor
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Biliary Obstruction ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Management Options ◽  
Extrahepatic Biliary Obstruction

The purpose of the present review is to discuss the diagnosis and management of cholestatic liver diseases. Differential diagnoses to consider are described, including causes of extrahepatic biliary obstruction such as gallstones, strictures, extrabiliary malignancies and pancreatitis. In addition, diseases that cause intrahepatic cholestasis such as primary biliary cirrhosis, primary sclerosing cholangitis, hepatocellular diseases and a variety of miscellaneous causes including drugs that may cause cholestasis are discussed. Primary biliary cirrhosis and primary sclerosing cholangitis are reviewed in detail, and management options are identified. The prognosis of patients with these diseases is discussed, and the Mayo Mathematical Models in Cholestatic Liver Disease for both primary biliary cirrhosis and primary sclerosing cholangitis are provided. Finally, management options for the complications of cholestasis are provided.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

scholarly journals Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

2020 ◽  
Vol 25 (2) ◽  
pp. 107-117
Author(s):  
Dong-Won Ahn
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Acute Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases, in which liver transplantation is required ultimately in most patients. Imaging studies such as magnetic resonance cholangiopancreatography have important role in diagnosis in most cases of PSC. PSC is usually accompanied by inflammatory bowel disease and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in endstage biliary cirrhosis requiring liver transplantation. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC and risk stratification is important to ensure all patients receive a personalised approach to their care. Medical therapy using ursodeoxycholic acid (UDCA) or obeticholic acid (OCA) has an important role to reduce the progression to end-stage liver disease in PBC.

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

2017 ◽  
Vol 37 (04) ◽  
pp. 305-313 ◽  
Author(s):  
Cynthia Levy ◽  
Eric Martin
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Graft Survival ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Immune Mediated ◽  
End Stage ◽  
Patient And Graft Survival

AbstractPrimary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

scholarly journals A Review of the Physiological and Immunological Functions of Biliary Epithelial Cells: Targets for Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis and Drug-induced Ductopenias

2004 ◽  
Vol 11 (3-4) ◽  
pp. 205-213 ◽  
Author(s):  
Chih-Te Wu ◽  
Paul A. Davis ◽  
VelImir A. Luketic ◽  
M. Eric Gershwin
Keyword(s):  
Epithelial Cells ◽  
Primary Biliary Cirrhosis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Tissue Injury ◽  
Cell Interaction ◽  
Biliary Cirrhosis ◽  
Drug Induced ◽  
Biliary Epithelial Cells ◽  
Immunological Diseases

Our understanding of biliary epithelial cells (BEC) in physiobiology and immunology has steadily expanded. BEC transports IgA as well as IgM into bile, synthesizes and secretes various chemokines, cytokines, and expresses adhesion molecules involved in cell interaction and signal transduction. These then suggest a myriad of potential roles for BEC in defense from invading microorganisms as well as the pathogenesis of diverse immunologically driven diseases such as primary biliary cirrhosis (PBC), graft-versus-host disease, and primary sclerosing cholangitis (PSC). Despite the progress, there still remain many areas of BEC biology that require further investigation. Most importantly, it remains to be clarified that the extent to which the immunologic activities observed in BEC represent a BEC response to tissue injury or whether BEC themselves are the active participants in the pathogenesis of various cholestatic immunological diseases, including PBC and PSC.

Human leukocyte antigen associations in Finnish liver transplantations due to primary sclerosing cholangitis and primary biliary cirrhosis

Open Medicine ◽  
2007 ◽  
Vol 2 (1) ◽  
pp. 12-25
Author(s):  
Irma Matinlauri ◽  
Markku Nurminen ◽  
Krister Höckerstedt ◽  
Helena Isoniemi
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Human Leukocyte Antigen ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Human Leukocyte ◽  
Positive Association ◽  
Biliary Cirrhosis ◽  
Leukocyte Antigen ◽  
Hla Dr3

AbstractA genetic predisposition has been suggested in primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC). The aim of the study was to evaluate human leukocyte antigen (HLA) frequencies and HLA associations in Finnish PSC and PBC patients. The relative frequencies of HLA-A,-B, and-DR antigens were compared between patients with PSC (n=50), or PBC (n=89), transplanted due to end-stage liver disease, and healthy members in the Finnish bone marrow donor registry (n=10000). Prevalence differences, prevalence ratios and the associated large-sample significance probabilities (2-sided P-values) and 95% confidence intervals were calculated.We found a strong positive association between PSC and HLA-B8 and-DR3, and a weak positive association between HLA-A1 and PSC. HLA-DR3 also had a weak positive association with PBC, and a weak negative association between HLA-DR5 and PBC was found. In conclusion, HLA-B8, and-DR3 are susceptible for progressive liver disease in PSC, and HLA-DR3 may also be susceptible for disease progression in PBC. HLA-DR5 may be protective against severe PBC.

Autoimmunity in liver disease

2013 ◽  
pp. 223-230
Author(s):  
Gavin P Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Disease Progression ◽  
Older Women ◽  
Primary Sclerosing Cholangitis ◽  
Clinical Features ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

Primary biliary cirrhosis (PBC) Autoimmune hepatitis Primary sclerosing cholangitis • PBC is a disease of older women (90% of patients are female). • Clinical features include initially: • profound fatigue starting in the prodrome • intense itch • arthralgia. • With disease progression: • hepatosplenomegaly...

Primary Biliary Cirrhosis

2015 ◽  
Author(s):  
Daniel S. Pratt ◽  
Lindsay Y. King
Keyword(s):  
Differential Diagnosis ◽  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Liver Diseases ◽  
American Association ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Common Cause ◽  
History Of

Primary biliary cirrhosis (PBC) is a progressive autoimmune disease of the liver. It is the most common cause of chronic intrahepatic cholestatic liver disease in adults. This review addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of PBC. Figures show the pathogenesis and natural history of PBC and histologic features of the four stages of PBC. Tables list diagnostic criteria for PBC via the American Association for the Study of Liver Diseases, differential diagnosis for PBC, medications used to treat PBC, secondary therapy for PBC, and follow-up of patients with PBC. This review contains 2 highly rendered figures, 5 tables, and 45 references.

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