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Role of novel retroviruses in chronic liver disease: assessing the link of human betaretrovirus with primary biliary cirrhosis.

ABSTRACT: A human betaretrovirus resembling mouse mammary tumor virus has been characterized in patients with primary biliary cirrhosis. The agent triggers a disease-specific phenotype in vitro with aberrant cell-surface expression of mitochondrial antigens. The presentation of a usually sequestered self-protein is thought to lead to the loss of tolerance and the production of anti-mitochondrial antibodies associated with the disease. Similar observations have been made in mouse models, where mouse mammary tumor virus infection has been linked with the development of cholangitis and production of anti-mitochondrial antibodies. The use of combination antiretroviral therapy has been shown to impact on histological and biochemical disease in mouse models of autoimmune biliary disease and in clinical trials of patients with primary biliary cirrhosis. However, the HIV protease inhibitors are not well tolerated in patients with primary biliary cirrhosis, and more efficacious regimens will be required to clearly link reduction of viral load with improvement of cholangitis.

SUBMITTER: Sharon D

PROVIDER: S-EPMC4353873 | biostudies-literature | 2015 Feb

REPOSITORIES: biostudies-literature

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Role of novel retroviruses in chronic liver disease: assessing the link of human betaretrovirus with primary biliary cirrhosis.

Sharon David D Mason Andrew L AL

Current infectious disease reports 20150201 2

A human betaretrovirus resembling mouse mammary tumor virus has been characterized in patients with primary biliary cirrhosis. The agent triggers a disease-specific phenotype in vitro with aberrant cell-surface expression of mitochondrial antigens. The presentation of a usually sequestered self-protein is thought to lead to the loss of tolerance and the production of anti-mitochondrial antibodies associated with the disease. Similar observations have been made in mouse models, where mouse mammar ...[more]

PMID: 25754451

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Project description:Primary biliary cirrhosis (PBC) is an immune-mediated chronic cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis and cirrhosis of the liver and may need liver transplantation in the late stage of disease. PBC primarily affects women (female preponderance 9-10:1) with a prevalence of up to 1 in 1,000 women over 40 years of age. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The diagnosis is based on sustained elevation of serum markers of cholestasis, i.e., alkaline phosphatase and gamma-glutamyl transferase, and the presence of serum antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex. Histologically, PBC is characterized by florid bile duct lesions with damage to biliary epithelial cells, an often dense portal inflammatory infiltrate and progressive loss of small intrahepatic bile ducts. Although the insight into pathogenetic aspects of PBC has grown enormously during the recent decade and numerous genetic, environmental, and infectious factors have been disclosed which may contribute to the development of PBC, the precise pathogenesis remains enigmatic. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at adequate doses of 13-15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. The mode of action of UDCA is still under discussion, but stimulation of impaired hepatocellular and cholangiocellular secretion, detoxification of bile, and antiapoptotic effects may represent key mechanisms. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarizes current knowledge on the clinical, diagnostic, pathogenetic, and therapeutic aspects of PBC.

Dataset Information

Role of novel retroviruses in chronic liver disease: assessing the link of human betaretrovirus with primary biliary cirrhosis.

Publications

Role of novel retroviruses in chronic liver disease: assessing the link of human betaretrovirus with primary biliary cirrhosis.

Similar Datasets

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