Primary biliary cirrhosis

2010 ◽  
pp. 2464-2468
Author(s):  
M.F. Bassendine ◽  
D.E.J. Jones
Keyword(s):  
Liver Disease ◽  
Epithelial Cells ◽  
Primary Biliary Cirrhosis ◽  
Case History ◽  
Bile Ducts ◽  
Cholestatic Liver Disease ◽  
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....

scholarly journals Primary biliary cirrhosis and myopathy: an uncommon association

1999 ◽  
Vol 54 (5) ◽  
pp. 165-168 ◽  
Author(s):  
Bruno Cupertino Migueletto ◽  
Abrahão Elias Hallack Neto ◽  
Elaine Zamora Domingues ◽  
Pedro Paulo Neves de Castro ◽  
Hartmut Stocker ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Bile Ducts ◽  
Cholestatic Liver Disease ◽  
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.

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.

T-Cell Autoimmunity in Primary Biliary Cirrhosis

1996 ◽  
Vol 91 (5) ◽  
pp. 551-558 ◽  
Author(s):  
David E. J. Jones
Keyword(s):  
T Cells ◽  
Liver Disease ◽  
T Cell ◽  
Primary Biliary Cirrhosis ◽  
Portal Tract ◽  
Cholestatic Liver Disease ◽  
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.

scholarly journals Primary Biliary Cirrhosis: Family Stories

2011 ◽  
Vol 2011 ◽  
pp. 1-11 ◽  
Author(s):  
Daniel Smyk ◽  
Evangelos Cholongitas ◽  
Stephen Kriese ◽  
Eirini I. Rigopoulou ◽  
Dimitrios P. Bogdanos
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Monozygotic Twins ◽  
Twin Studies ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Family Stories ◽  
Immune Mediated ◽  
Increased Risk ◽  
Environmental Component ◽  
High Concordance

Primary biliary cirrhosis (PBC) is a chronic immune-mediated cholestatic liver disease of unknown aetiology which affects mostly women in middle age. Familial PBC is when PBC affects more than one member of the same family, and data suggest that first-degree relatives of PBC patients have an increased risk of developing the disease. Most often, these familial clusters involve mother-daughter pairs, which is consistent with the female preponderance of the disease. These clusters provide evidence towards a genetic basis underlying PBC. However, clusters of nonrelated individuals have also been reported, giving strength to an environmental component. Twin studies have demonstrated a high concordance for PBC in monozygotic twins and a low concordance among dizygotic twins. In conclusion, studies of PBC in families clearly demonstrate that genetic, epigenetic, and environmental factors play a role in the development of the disease.

scholarly journals E. coliInduced Experimental Model of Primary Biliary Cirrhosis: At Last

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Andreas L. Koutsoumpas ◽  
Daniel S. Smyk ◽  
Dimitrios P. Bogdanos
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Infected Animal ◽  
Infectious Agent ◽  
Epidemiological Studies ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
E Coli ◽  
Intrahepatic Bile Ducts ◽  
Additional Support ◽  
Tract Infections

Recurrent urinary tract infections (UTI) have been considered potential triggers of primary biliary cirrhosis (PBC), an autoimmune cholestatic liver disease characterised by progressive destruction of intrahepatic bile ducts. Additional support for the link made between PBC and UTI was based on early observations of recurrent episodes of bacteriuria in female patients with PBC. A series of large epidemiological studies demonstrated a strong correlation between recurrent UTI and PBC, initiating a series of studies investigating the role ofEscherichia coli(E. coli, the most prevalent organism isolated in women with UTI) as a trigger of PBC. Immunological evidence of B- and T-cell cross-reactive responses implicating PBC-specific autoantigens andE. colimimics have been clearly demonstrated, adding support to the notion thatE. coliis a potential infectious inducer of PBC in susceptible individuals. One of the major limitations in proving theE. coli/PBC association was the lack of reliableE. coli-infected animal models of PBC. This review provides an overview of the evidence linking this infectious agent with PBC and discusses the pros and cons of a recently developedE. coli-infected animal model of PBC.

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.

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