Primary Biliary Cirrhosis

Liver Diseases ◽

American Association ◽

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.

Medical Management of Chronic Cholestatic Liver Diseases

Canadian Journal of Gastroenterology ◽

10.1155/2000/871543 ◽

2000 ◽

Vol 14 (suppl d) ◽

pp. 93D-98D ◽

Cited By ~ 1

Author(s):

Andrea A Gossard ◽

Keith D Lindor

Keyword(s):

Liver Disease ◽

Primary Sclerosing Cholangitis ◽

Liver Diseases ◽

Biliary Obstruction ◽

Sclerosing Cholangitis ◽

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.

Primary biliary cirrhosis and myopathy: an uncommon association

Revista do Hospital das Clínicas ◽

10.1590/s0041-87811999000500007 ◽

1999 ◽

Vol 54 (5) ◽

pp. 165-168 ◽

Cited By ~ 5

Author(s):

Bruno Cupertino Migueletto ◽

Abrahão Elias Hallack Neto ◽

Elaine Zamora Domingues ◽

Pedro Paulo Neves de Castro ◽

Hartmut Stocker ◽

...

Keyword(s):

Liver Disease ◽

Bile Ducts ◽

Biliary Cirrhosis ◽

Muscular Weakness ◽

Muscular Diseases ◽

Histological Features ◽

Intrahepatic Bile Ducts ◽

Organ Systems

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

T-Cell Autoimmunity in Primary Biliary Cirrhosis

Clinical Science ◽

10.1042/cs0910551 ◽

1996 ◽

Vol 91 (5) ◽

pp. 551-558 ◽

Cited By ~ 10

Author(s):

David E. J. Jones

Keyword(s):

T Cells ◽

Liver Disease ◽

T Cell ◽

Portal Tract ◽

Biliary Cirrhosis ◽

Early Stage Disease ◽

Control Subjects ◽

Autoreactive Cells

1. Primary biliary cirrhosis is a chronic cholestatic liver disease with an autoimmune aetiology. The classical histopathological lesion, of portal tract biliary epithelial cell damage, is accompanied by a T-cell-rich mononuclear cell infiltrate and upregulation of cell surface markers suggestive of local T-cell activation and cytokine release. This suggests that T-cell mediated mechanisms play an important role in tissue damage in primary biliary cirrhosis. 2. CD4+ T-cells specific for the E2 component of human pyruvate dehydrogenase complex (PDC-E2), a highly conserved enzyme which plays a critical role in intermediate metabolism, are present in the peripheral repertoire of the majority of patients with primary biliary cirrhosis. These cells are almost entirely absent from normal and chronic liver disease control subjects. The observations that peripheral blood PDC-E2-specific cells are most commonly seen in early stage disease, when active bile duct damage is occurring, and that PDC-E2-specific cells can be found in the portal tract infiltrate at times when this damage is occurring, suggest that these autoreactive cells may have a role to play in the aetiology of primary biliary cirrhosis. 3. T-cells specific for the whole PDC and its E1 component are seen in significant numbers of normal control subjects as well as patients with primary biliary cirrhosis. Retention of potentially autoreactive cells in the normal T-cell repertoire has been reported for a number of other autoantigens. 4. T-cell epitopes appear to be widely distributed throughout PDC-E2. This is in contrast to the B-cell epitopes which are highly restricted to the inner lipoyl binding domain of the protein.

Ultrastructural Aspects of Primary Biliary Cirrhosis and Other Types of Cholestatic Liver Disease

Gastroenterology ◽

10.1016/s0016-5085(19)32737-4 ◽

1974 ◽

Vol 67 (5) ◽

pp. 858-869 ◽

Cited By ~ 30

Author(s):

Antonio Chedid ◽

Mitchell A. Spellberg ◽

Roy A. DeBeer

Keyword(s):

Liver Disease ◽

Primary Biliary Cirrhosis as a Rare Cause of Severe Cholestatic Liver Disease in an African Male

The American Journal of Gastroenterology ◽

10.14309/00000434-201110002-00570 ◽

2011 ◽

Vol 106 ◽

pp. S218-S219

Author(s):

Nihar Shah ◽

Hamid Shaaban

Keyword(s):

Liver Disease ◽

Autoimmunity in liver disease

Oxford Handbook of Clinical Immunology and Allergy ◽

10.1093/med/9780198789529.003.0008 ◽

2019 ◽

pp. 245-252

Author(s):

Gavin Spickett

Keyword(s):

Liver Disease ◽

Autoimmune Hepatitis ◽

Primary Sclerosing Cholangitis ◽

Liver Diseases ◽

Sclerosing Cholangitis ◽

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.

Primary biliary cirrhosis

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.152103_update_001 ◽

2010 ◽

pp. 2464-2468

Author(s):

M.F. Bassendine ◽

D.E.J. Jones

Keyword(s):

Liver Disease ◽

Epithelial Cells ◽

Case History ◽

Bile Ducts ◽

Biliary Cirrhosis ◽

Blood Tests ◽

Intrahepatic Bile Ducts ◽

Immune Mediated

Case History—A 60 yr old woman presenting with abnormal liver blood tests. Primary biliary cirrhosis is a chronic, cholestatic liver disease in which the biliary epithelial cells lining the small intrahepatic bile ducts are the target for immune-mediated damage leading to progressive ductopenia. The cause is unknown, but presumed to be autoimmune....

Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

The Korean Journal of Pancreas and Biliary Tract ◽

10.15279/kpba.2020.25.2.107 ◽

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 ◽

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.