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Primary biliary cirrhosis.

Kumagi T, Heathcote EJ - Orphanet J Rare Dis (2008)

Bottom Line: PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease.Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years.Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Toronto Western Hospital (University Health Network/University of Toronto), Toronto, Ontario, Canada. masato_teru@yahoo.co.jp

ABSTRACT
Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.

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Primary biliary cirrhosis, showing ductular proliferation (stage 2 of Scheuer's classification). Small ductular structures (arrow) is shown in a portal tract (asterisk). (Hematoxylin-eosin.)
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Figure 2: Primary biliary cirrhosis, showing ductular proliferation (stage 2 of Scheuer's classification). Small ductular structures (arrow) is shown in a portal tract (asterisk). (Hematoxylin-eosin.)

Mentions: The characteristic lesion of PBC is the asymmetric destruction of the intralobular bile ducts within portal triads. Histological staging systems of PBC have been proposed and they have been divided into 3 or 4 distinct stages, according to the degree of fibrosis, inflammation and/or bile duct damage [4,35-37]. According to the Ludwig's classification [37], stage 1 is defined by portal inflammation. Stage 2 is extension of this inflammation beyond portal tracts into the surrounding parenchyma, with or without associated duct loss. In stage 3, fibrous septa link adjacent portal triads. Stage 4 represents cirrhosis. On the other hand, with Scheuer's system [35], stage 1 is referred to as the florid duct lesion or chronic non-suppurative destructive cholangitis (Figure 1). In stage 2, there is proliferation of the small bile ductules (Figure 2). Stage 3 is characterized by fibrosis or scarring. Stage 4 is cirrhosis. However, the entire liver is not always uniformly involved, and a single biopsy may demonstrate the feature of all four stages simultaneously. Thus both under- and over-estimation may occur. It is crucial to have a sufficient size of specimen to minimize error [38]. There should be a minimum of 10 portal tracts visualized before a confident diagnosis of bile duct loss can be established. Although liver biopsy is not always necessary to make a diagnosis of PBC, it does allow the severity of disease to be clarified. It also may indicate the need of specific therapeutic regimens or management (such as ultrasound screening for hepatocellular carcinoma, HCC, in PBC patients found to be cirrhotic) and, additionally, may be used as an objective way for evaluating the response to treatment.


Primary biliary cirrhosis.

Kumagi T, Heathcote EJ - Orphanet J Rare Dis (2008)

Primary biliary cirrhosis, showing ductular proliferation (stage 2 of Scheuer's classification). Small ductular structures (arrow) is shown in a portal tract (asterisk). (Hematoxylin-eosin.)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2266722&req=5

Figure 2: Primary biliary cirrhosis, showing ductular proliferation (stage 2 of Scheuer's classification). Small ductular structures (arrow) is shown in a portal tract (asterisk). (Hematoxylin-eosin.)
Mentions: The characteristic lesion of PBC is the asymmetric destruction of the intralobular bile ducts within portal triads. Histological staging systems of PBC have been proposed and they have been divided into 3 or 4 distinct stages, according to the degree of fibrosis, inflammation and/or bile duct damage [4,35-37]. According to the Ludwig's classification [37], stage 1 is defined by portal inflammation. Stage 2 is extension of this inflammation beyond portal tracts into the surrounding parenchyma, with or without associated duct loss. In stage 3, fibrous septa link adjacent portal triads. Stage 4 represents cirrhosis. On the other hand, with Scheuer's system [35], stage 1 is referred to as the florid duct lesion or chronic non-suppurative destructive cholangitis (Figure 1). In stage 2, there is proliferation of the small bile ductules (Figure 2). Stage 3 is characterized by fibrosis or scarring. Stage 4 is cirrhosis. However, the entire liver is not always uniformly involved, and a single biopsy may demonstrate the feature of all four stages simultaneously. Thus both under- and over-estimation may occur. It is crucial to have a sufficient size of specimen to minimize error [38]. There should be a minimum of 10 portal tracts visualized before a confident diagnosis of bile duct loss can be established. Although liver biopsy is not always necessary to make a diagnosis of PBC, it does allow the severity of disease to be clarified. It also may indicate the need of specific therapeutic regimens or management (such as ultrasound screening for hepatocellular carcinoma, HCC, in PBC patients found to be cirrhotic) and, additionally, may be used as an objective way for evaluating the response to treatment.

Bottom Line: PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease.Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years.Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Medicine, Toronto Western Hospital (University Health Network/University of Toronto), Toronto, Ontario, Canada. masato_teru@yahoo.co.jp

ABSTRACT
Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.

Show MeSH
Related in: MedlinePlus