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Hepatology (Baltimore, Md.) Jul 2009
Topics: Humans; Liver Cirrhosis, Biliary
PubMed: 19554543
DOI: 10.1002/hep.22906 -
Gastroenterology Clinics of North... Jun 2011Primary biliary cirrhosis is a chronic autoimmune inflammatory disease of the liver with a striking female preponderance. It has an insidious onset and typically affects... (Review)
Review
Primary biliary cirrhosis is a chronic autoimmune inflammatory disease of the liver with a striking female preponderance. It has an insidious onset and typically affects middle-aged women. The disease manifests gradually with symptoms of fatigue, pruritis, and increased alkaline phosphatase levels on laboratory evaluation. The hallmark of the disease is the circulating antimitochondrial antibody. Histology is characterized by inflammation of the bile ducts, destruction of cholangiocytes, and subsequent cholestasis, progressing to biliary cirrhosis. The standard treatment for primary biliary cirrhosis is ursodeoxycholic acid, which improves survival, but the disease can still lead to cirrhosis and liver failure over decades.
Topics: Female; Humans; Liver Cirrhosis, Biliary; Male; Sex Factors
PubMed: 21601785
DOI: 10.1016/j.gtc.2011.03.008 -
Lancet (London, England) Jul 2003Primary biliary cirrhosis is a chronic cholestatic liver disease of adults. This disorder is characterised histologically by chronic non-suppurative destruction of... (Review)
Review
Primary biliary cirrhosis is a chronic cholestatic liver disease of adults. This disorder is characterised histologically by chronic non-suppurative destruction of interlobular bile ducts leading to advanced fibrosis, cirrhosis, and liver failure. The precise aetiopathogenesis of primary biliary cirrhosis remains unknown, although dysregulation of the immune system and genetic susceptibility both seem to be important. Affected patients are typically middle-aged women with abnormal serum concentrations of alkaline phosphatase. Presence of antimitochondrial antibody in serum is almost diagnostic of the disorder. Identification of primary biliary cirrhosis is important, because effective treatment with ursodeoxycholic acid has been shown to halt disease progression and improve survival without need for liver transplantation. However, therapeutic options for disease-related complications-including fatigue and metabolic bone disease-remain unavailable. Mathematical models have been developed that accurately predict the natural history of primary biliary cirrhosis in individuals. Despite advances in understanding of the disease, it remains one of the major indications for liver transplantation worldwide.
Topics: Disease Progression; Female; Humans; Liver Cirrhosis, Biliary; Male; Prevalence
PubMed: 12853201
DOI: 10.1016/S0140-6736(03)13808-1 -
Gut Sep 1991
Review
Topics: Humans; Liver; Liver Cirrhosis, Biliary; Liver Transplantation; Prognosis
PubMed: 1916473
DOI: 10.1136/gut.32.suppl.s73 -
The New England Journal of Medicine Nov 1996
Review
Topics: Colchicine; Cyclosporine; Humans; Liver; Liver Cirrhosis, Biliary; Liver Transplantation; Methotrexate; Mitochondria, Liver; Prognosis; Ursodeoxycholic Acid
PubMed: 8900092
DOI: 10.1056/NEJM199611213352107 -
Orphanet Journal of Rare Diseases Jan 2008Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile... (Review)
Review
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.
Topics: Aged; Antibodies, Antinuclear; Autoimmune Diseases; Cholagogues and Choleretics; Female; Humans; Liver Cirrhosis, Biliary; Liver Transplantation; Male; Middle Aged; Sex Factors; Ursodeoxycholic Acid
PubMed: 18215315
DOI: 10.1186/1750-1172-3-1 -
Journal of Internal Medicine May 1997
Review
Topics: Autoimmune Diseases; Causality; Disease Progression; Genetic Predisposition to Disease; Humans; Immunotherapy; Liver Cirrhosis, Biliary; Liver Transplantation
PubMed: 9183301
DOI: 10.1046/j.1365-2796.1997.127138000.x -
Seminars in Liver Disease Sep 2016Primary biliary cirrhosis is a rather uncommon, slowly progressive, cholestatic liver disease that predominantly affects middle-aged women. Apart from the changes in the... (Review)
Review
Primary biliary cirrhosis is a rather uncommon, slowly progressive, cholestatic liver disease that predominantly affects middle-aged women. Apart from the changes in the gut microbiome that have been described in liver disease in general, little is known of the composition of the microbiome in primary biliary cirrhosis. Nevertheless, epidemiological, clinical, and some experimental evidence points to the possible role of a bacterium (or bacteria) in the initiation of the autoimmune process that leads to the development of this unique clinical phenotype.
Topics: Carcinoma, Hepatocellular; Case-Control Studies; Female; Gastrointestinal Microbiome; Humans; Liver Cirrhosis, Biliary; Liver Neoplasms; Phenotype
PubMed: 27997975
DOI: 10.1055/s-0036-1594006 -
Best Practice & Research. Clinical... Oct 2010Primary biliary cirrhosis (PBC) is an idiopathic chronic autoimmune liver disease that primarily affects women. It is believed that the aetiology for PBC is a... (Review)
Review
Primary biliary cirrhosis (PBC) is an idiopathic chronic autoimmune liver disease that primarily affects women. It is believed that the aetiology for PBC is a combination between environmental triggers in genetically vulnerable persons. The diagnosis for PBC is made when two of the three criteria are fulfilled and they are: (1) biochemical evidence of cholestatic liver disease for at least 6 month's duration; (2) anti-mitochondrial antibody (AMA) positivity; and (3) histologic features of PBC on liver biopsy. Ursodeoxycholic acid (UDCA) is the only FDA-approved medical treatment for PBC and should be administered at a recommended dose of 13-15 mg/kg/day. Unfortunately despite adequate dosing of UDCA, approximately one-third of patients does not respond adequately and may require liver transplantation. Future studies are necessary to elucidate the role of environmental exposures and overall genetic impact not only in the development of PBC, but on disease progression and variable clinical response to therapy.
Topics: Animals; Disease Management; Disease Progression; Environmental Exposure; Humans; Liver; Liver Cirrhosis, Biliary; Liver Transplantation; Major Histocompatibility Complex; Mitochondria, Liver; Treatment Failure; Ursodeoxycholic Acid
PubMed: 20955967
DOI: 10.1016/j.bpg.2010.07.006 -
Digestive Diseases (Basel, Switzerland) 1994Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged... (Review)
Review
Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged women. It is nearly always associated with an antibody directed against a component of the pyruvate dehydrogenase complex located on the inner wall of the mitochondria. The disease is associated with a number of other associated autoimmune disorders. No totally effective medical treatment has been established for the disease, although urosdeoxycholic acid appears promising. Complications of cholestasis such as fat malabsorption and fat-soluble vitamin deficiency should be excluded or corrected when found. Individual patient prognosis varies. Several models for estimating individual patient survival are available. Liver transplantation is recognized as a procedure to extend and improve the quality of life for patients with advanced disease.
Topics: Female; Humans; Immunosuppressive Agents; Liver; Liver Cirrhosis, Biliary; Liver Transplantation
PubMed: 7712616
DOI: 10.1159/000171469