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Antimitochondrial Antibody-Negative Primary Biliary Cirrhosis. Gastroenterology Clinics of North America. 37(2): 479-484. June 2008.

This article about antimitochondrial antibody-negative primary biliary cirrhosis (PBC) is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. The authors describe PBC as a chronic cholestatic liver disease of unclear cause, characterized by nonsuppurative destruction of the bile ducts and serologically by the presence of antimitochondrial antibodies (AMA). The authors briefly review the controversy and uncertainty regarding AMA-positive and AMA-negative types of PBC, noting that recent developments strengthen the idea that they are truly one condition. The authors review the clinical, biochemical, serologic, and histopathologic features and treatment approach and outcomes in patients who have AMA-negative PBC. The authors conclude that AMA-negative PBC shares similar clinical, biochemical, histologic, and prognostic features with classic PBC. Management of AMA-negative PBC should not differ from treatment of AMA-positive disease. 1 figure. 1 table. 20 references.

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Celiac Disease and Autoimmunity in the Gut and Elsewhere. Gastroenterology Clinics of North America. 37(2): 411-428. June 2008.

This article about celiac disease and autoimmunity in the gut and elsewhere is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. Celiac disease is a common immune-mediated enteropathy characterized by sensitivity to the wheat protein, gluten. The authors note that celiac disease is often difficult to diagnose due in large part to the silent form of the disease that affects the majority of patients. Overall mild clinical symptoms with nonspecific complaints such as fatigue, headaches, and arthralgias are common and can delay diagnosis. This article discusses the gut immunogenesis of celiac disease, the role of environmental factors, and the risk of autoimmune disease. The authors focus on the autoimmune connective tissue diseases, endocrine, and dermatologic conditions associated with celiac disease, as well as the related gut inflammatory disorders of refractory celiac disease, autoimmune enteropathy, collagenous enteritis, and collagenous colitis. Specific diseases discussed include Sjogren's syndrome, inflammatory arthritis, Addison's disease, autoimmune insulin-dependent diabetes mellitus (AIDDM), IgA deficiency, pernicious anemia, primary biliary cirrhosis, and autoimmune hepatitis. The authors conclude with a brief discussion of screening recommendations in patient populations with these immune-related illnesses. 1 figure. 2 tables. 79 references.

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Cholestasis Post Liver Transplantation. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 171-182.

This chapter on cholestasis that occurs after liver transplantation is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The chapter covers biliary complications, preservation or reperfusion injury and ABO incompatibility, small-for-size syndrome, hepatic artery thrombosis, infectious complications, drug-induced acute cellular rejection, chronic rejection, and recurrent disease, including primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and viral hepatitis. The authors caution that cholestasis can occur anytime throughout the posttransplant period, may be intrahepatic or extrahepatic in origin, and has a very broad differential diagnosis. Careful diagnostic imaging of the biliary tree is an important first step in the workup, followed by liver biopsy if clinically indicated. 1 table. 50 references.

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Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. 188 p.

This book offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The text includes 10 chapters: the diagnosis of cholestasis, drug-induced cholestasis, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), overlap syndrome with autoimmune hepatitis, rare causes of cholestasis, cholestatic variants of viral disease and alcohol, cholestasis from systemic disorders, complications of cholestasis, and posttransplantation cholestasis. Specific topics include liver tests and antibodies, cross-sectional studies, liver biopsy, other diagnostic tests, endoscopic ultrasound, herbal remedies, the risk of colon cancer with inflammatory bowel disease (IBD), colon cancer posttransplant, and cholangiocarcinoma, genetic disorders, viral hepatitis, sarcoid disease, lymphoma, granulomatous disease, cystic fibrosis, rheumatologic diseases, osteoporosis, pruritus, hyperlipidemia, strictures, viral disease, and recurrent cholestatic liver disease. Each chapter begins with a brief outline and concludes with a summary and a list of references. The text concludes with a subject index.

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Cholestatic Liver Disease: Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and AIDS-Associated Cholangiopathy. IN: Hauser, S., ed. Mayo Clinic Gastroenterology and Hepatology Board Review. 3rd ed. New York, NY: Informa Healthcare USA. 2008. pp 377-382.

This chapter on cholestatic liver disease is from a comprehensive textbook that provides an in-depth examination of essential knowledge in gastroenterology, hepatology, and the related areas of pathology, endoscopy, nutrition, and radiology. This chapter covers primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and AIDS-associated cholangiopathy. The author notes that drug-induced cholestasis may be the most common explanation for cholestasis in patients without biliary obstruction. The author briefly reviews the differential diagnosis. The author outlines the recommended treatment for each of these conditions, including the management of the complications of cholestasis, such as malabsorption, pruritus, and bone disease. PBC involves women in 90 percent of cases and is characterized by antimitochondrial antibodies, fatigue, and pruritus. Treatment usually involves ursodiol for patients at any stage of PBC who have abnormal findings on liver tests. Ursodiol improves survival free of transplantation, decreases the risk of the development of cirrhosis and varices, and lowers lipid levels. PSC is the next most common cholestatic condition in adults. About 70 percent of these patients have inflammatory bowel disease. Unfortunately, no effective therapy is available for PSC. AIDS-associated cholangiopathy is defined as biliary obstruction due to infections that lead to biliary strictures. Treatment with highly active retroviral therapy decreases the percentage of patients with HIV infection that progresses to AIDS and may eventually prevent the development of biliary complications. 2 figures. 1 table. 26 references.

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Eosinophilic and Autoimmune Gastrointestinal Disease: New Insights and New Entities. Gastroenterology Clinics of North America. 37(2): 1-492. June 2008.

This issue of Gastroenterology Clinics of North America focuses on eosinophilic and autoimmune gastrointestinal disease. Eosinophilic esophagitis (EE) is a clinicopathologic disease characterized by upper intestinal symptoms and the finding of more than 15 or 20 eosinophils in the esophageal epithelium; these findings are unresponsive to proton pump inhibitor treatment. Eosinophils are a type of white blood cell. EE is a common disease that affects both children and adults. This issue offers 11 articles covering gut eosinophilia in food allergy and in systemic and autoimmune diseases, eosinophilic gastroenteritis in adults, the clinical manifestations of eosinophilic esophagitis in children, functional gastrointestinal disorders and the potential role of eosinophils, enteric autoantibodies and gut motility disorders, celiac disease and autoimmunity in the gut and elsewhere, autoantibodies in inflammatory bowel disease (IBD), autoimmune pancreatitis, the diagnosis and treatment of autoimmune hepatitis, and antimitochondrial antibody-negative primary biliary cirrhosis (PBC). Each article is written by experts in the field and concludes with a list of references for readers seeking more information. A subject index concludes the volume.

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Overlap Syndromes with Autoimmune Hepatitis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 85-104.

This chapter on overlap syndromes with autoimmune hepatitis is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The author of this chapter notes that autoimmune hepatitis, or some features thereof, may coexist with primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). The overlapping symptoms can include significant elevations of serum aminotransferase, immunoglobulin G, and total protein concentrations, additional circulating antibiotics, and intense, plasma-cell-predominant interface inflammation––hepatitis––that is responsive to systemic immunosuppressive therapy. The chapter covers nomenclature, the interplay of PBC and autoimmune hepatitis, and the overlap between PSC and autoimmune hepatitis. The author concludes that although there is a lot of debate about how autoimmune overlap syndromes should be defined, their existence is not in doubt. The chapter is illustrated with black-and-white photographs. 3 figures. 4 tables. 74 references.

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Primary Biliary Cirrhosis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 45-66.

This chapter on primary biliary cirrhosis (PBC) is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. Primary biliary cirrhosis is a chronic progressive cholestatic liver disease that primarily affects middle-aged women. The chapter covers pathogenesis, epidemiology, clinical features, diagnosis, treatment, natural history and prognosis, and liver transplantation for PBC. The authors note that the pathogenesis of this disease is unknown, but some research points to genetic and environmental factors that may initiate the autoimmune process. Patients are often asymptomatic at the time of their diagnosis, but as inflammation destroys the bile ducts and fibrosis develops, symptoms of fatigue and pruritus may become present. Ursodeoxycholic acid (UDCA) is the only recommended treatment and may improve liver biochemistries, delay progression of fibrosis and development of esophageal varices, and may improve survival in selected patients. Liver transplantation is the only definitive therapy once end-stage liver disease occurs. 1 table. 153 references.

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Liver Diseases in Pregnancy. IN: Pregnancy in Gastrointestinal Diseases. 2nd ed. Bethesda, MD: American College of Gastroenterology. 2007. pp 32-53.

This chapter about liver diseases in pregnancy is from a monograph that presents updated information about pregnancy in women with gastrointestinal disorders. The authors stress that a complete understanding of the physiological changes that affect pregnancy and of the different liver diseases that occur during pregnancy is essential for early recognition and management of pregnancy-associated liver disorders. The chapter focuses on bringing readers up to date on the research in the area covered, the recommended treatments, and patient management concerns, notably issues of maternal and fetal safety. Separate sections discuss the physiological changes that affect pregnancy, diagnostic imaging tests used in pregnancy, liver disorders that are exclusive or unique to pregnancy, liver diseases that may occur during pregnancy or intercurrent liver diseases in pregnancy, and changes that occur when a woman with a pre-existing liver disease becomes pregnant. Specific conditions discussed include hepatic involvement in hyperemesis gravidarum, acute fatty liver of pregnancy (AFLP), intrahepatic cholestasis of pregnancy (IHCP), hemolysis, elevated liver enzymes and low platelets syndrome (HELLP syndrome), viral hepatitis, HIV infection, herpes simplex viral infections, cytomegalovirus infection (CMV), alcohol use, portal hypertension, autoimmune hepatitis (AIH), Wilson disease, primary biliary cirrhosis and primary sclerosing cholangitis, Budd-Chiari syndrome, gallstone disease in pregnancy, and liver transplant. The authors conclude that preventive measures, including early prenatal care, avoidance of risky behaviors that could increase a woman’s chance of acquiring infections, and cessation of smoking and drinking alcohol are vital in decreasing morbidity and mortality in pregnancy. Although liver disease in pregnancy is associated with an increased risk for morbidity and mortality, clinical outcomes have improved for both mother and baby, and pregnancy is not contraindicated in patients with liver disease or in patients who have had a liver transplant. 3 tables. 54 references.

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Primary Biliary Cirrhosis. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2007. 2 p.

This fact sheet reviews primary biliary cirrhosis, a disease that slowly destroys the liver’s bile ducts. When the ducts are damaged, bile builds up in the liver and damages liver tissue. The fact sheet covers the symptoms, diagnosis, and treatment options for primary biliary cirrhosis (PBC). Initial treatment for PBC is usually aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed. The fact sheet concludes with the contact information for the American Liver Foundation and a brief description of the activities of the National Digestive Diseases Information Clearinghouse (NDDIC).

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Clinical Utility of Biomarkers of Liver Fibrosis. Gastroenterology and Hepatology. 2(1): 48-57. January 2006.

This article explores the clinical use of biomarkers of liver fibrosis. Liver fibrosis leads to impaired function, portal hypertension, and reduced survival. Thus an accurate evaluation of liver fibrosis in chronic liver disease is important to determine prognosis, therapy outcomes, and disease progression. The traditional method, needle liver biopsy, is an invasive procedure that is associated with small sample size and inaccurate staging, and provides only a semi-quantitative assessment of fibrosis. The authors review a number of simple and specific extracellular matrix biochemical markers that are predictive of fibrosis in patients with chronic liver disease. In patients with hepatitis C, serum HA has been shown to reflect virus response to therapy. Serum HA may also be predictive of severe complications in patients with compensated cirrhosis due to hepatitis C. Markers such as PIIINP and YKL-40 may also predict clinical outcomes in other chronic liver diseases such as primary biliary cirrhosis or alcoholic liver disease. The authors conclude by addressing concerns about overcoming the inherent issue of validation against a static and imperfect test such as liver biopsy; as with liver biopsy, noninvasive markers are imperfect. 4 tables. 78 references.

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Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. 356 p.

This user-friendly reference book provides gastroenterologists with an overview of the management of acute and chronic liver disease. The book offers 16 chapters: evaluation of the liver patient, cirrhosis and its complications, acute and chronic viral hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis, autoimmune hepatitis, nonalcoholic fatty liver disease, metabolic liver disease, vascular diseases involving the liver, benign and malignant tumors of the liver, liver disease in pregnancy, postoperative jaundice, nonviral infections of the liver, hepatopulmonary syndrome, portopulmonary hypertension, liver transplantation, and drug hepatotoxicity. Each chapter presents an introduction, consideration of etiology, epidemiology, clinical presentation and symptoms, risk factors, complications, and treatment approaches. The chapters include charts and figures and conclude with a list of references. Most chapters include patient care algorithms. A subject index concludes the volume.

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Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. 428 p.

This textbook provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The book was written by a multidisciplinary panel of international experts with extensive experience in this population of patients. The book offers 23 chapters in six sections: anatomy, pathophysiology, and epidemiology of the biliary system; diagnostic and therapeutic approaches for the biliary tree and gallbladder; specific conditions; the intrahepatic and extrahepatic bile ducts; intrahepatic cholestasis; and the pediatric population. Specific topics include noninvasive imaging, endoscopic diagnosis and treatment, percutaneous biliary imaging and intervention, radiation therapy, surgery, laparoscopic treatment, laparoscopic biliary injuries, treatment for biliary malignancies, the gallbladder, gallstones, acute cholangitis, cystic diseases of the biliary system, biliary complications of liver transplantation, primary sclerosing cholangitis, cholangiocarcinoma, primary biliary cirrhosis, and biliary disease in infants and children. Each chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. The book is illustrated with black-and-white photographs and line drawings; one section of color plates is included. The book concludes with the answers to the self-test study questions and a detailed subject index.

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Nutrition Concerns of the Patient with Primary Biliary Cirrhosis or Primary Sclerosing Cholangitis. Practical Gastroenterology. 30(4): 92-100. April 2006.

This article reviews the nutritional concerns of the patient with primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC), two cholestatic liver diseases that are characterized by impairment of the bile flow. The primary nutritional consequences of cholestasis are related to malabsorption caused by lack of sufficient bile acids in the intestinal lumen. Many digestive enzymatic reactions are affected by impaired bile flow into the small bowel. This decrease in biliary excretion of cholesterol and other lipids in cholestasis often lead to hyperlipidemia. The authors provide an overview of the nutritional assessment, diagnosis, and management of patients with chronic cholestatic liver disorders, notably PBC, PSC, and autoimmune cholangiopathy. The authors discuss protein-caloric malnutrition, fat malabsorption, hyperlipidemia (primarily high cholesterol levels), and fat-soluble vitamins deficiency. The authors caution that sodium and water retention and other factors may interfere with an accurate nutritional evaluation in this patient population. However, the nutritional complications of severe and progressive cholestasis should be addressed and treated in a systematic manner in these patients. 5 tables. 8 references.

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Pathology of the Intrahepatic and Extrahepatic Bile Ducts and Gallbladder. IN: Clavien, P.; Baillie, J., eds. Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. pp 21-57.

This chapter on pathology is from a textbook that provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The author focuses on the pathology of the intrahepatic and extrahepatic bile ducts and gallbladder. The chapter lists the characteristic morphologic features of primary biliary cirrhosis (PBC), with an emphasis on the florid duct lesion, and describes the histopathologic staging schemes for PBC and primary sclerosing cholangitis (PSC). Other topics include the morphologic features of acute cellular rejection in the hepatic allograft, use of grading schemas for rejection of a transplanted liver, the use of liver biopsy and the characteristic morphologic features of PSC, the secondary causes of sclerosing cholangitis, fibropolycystic diseases of the liver, the anatomic and gross features of cholangiocarcinoma, the staging systems used for gallbladder cancer, and the precursor lesions and etiologic factors in gallbladder cancer. The chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. 25 figures. 10 tables. 122 references.

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Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 87-104.

This chapter about primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors define PBC as a chronic cholestatic, autoimmune liver disease that predominantly affects middle-aged women. Over time, fibrosis, cirrhosis, and complications of portal hypertension and cholestasis may develop in patients with PBC. Ursodeoxycholic acid has been shown to improve cholestatic liver-associated enzymes and to improve transplant-free survival, although the effect is not certain. Liver transplantation is the treatment of choice for patients with advanced PBC. Primary sclerosing cholangitis is a chronic cholestatic liver disease that primary affects young to middle-aged men, who typically have concurrent inflammatory bowel disease (IBD). PSC patients are at risk for bacterial cholangitis, cholangiocarcinoma, and complications of liver failure and cholestasis. Although there is no proven therapy specific for PSC, medical therapies should be directed toward managing the complications of portal hypertension and progressive cholestasis. Liver transplantation is the only option that has been clearly shown to improve patient survival. 5 tables. 20 references.

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Primary Biliary Cirrhosis. IN: Clavien, P.; Baillie, J., eds. Diseases of the Gallbladder and Bile Ducts: Diagnosis and Treatment. 2nd ed. Williston, VT: Blackwell Publishing Inc. 2006. pp 341-352.

This chapter on primary biliary cirrhosis (PBC) is from a textbook that provides a comprehensive and critical approach to both established and new diagnostic and therapeutic modalities for diseases of the gallbladder and bile ducts. The authors begin with a discussion of nomenclature, noting that health care providers must communicate clearly to patients the discrepancy between the name PBC and a patient’s actual clinical status. PBC is characterized by destruction of the interlobular bile ducts with duct invasion by chronic inflammatory cells and apoptosis of biliary epithelial cells, leading to progressive bile duct loss, fibrosis, and eventual cirrhosis. The authors describe the multifactorial etiology of PBC, the role of genetic factors in its development, the importance of early diagnosis even in asymptomatic patients, and the management of PBC and its complications. They note that no curative medical therapy for PBC exists; however, medications are available that slow down the progression of the disease and can relieve symptoms in the majority of patients. Some patients with PBC will develop end-stage liver failure and require liver transplantation. The chapter includes a summary of objectives, a list of suggested readings, extensive references, and a set of self-test questions that focus on the material covered in the chapter. 2 figures. 4 tables. 63 references.

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Evaluation of the Patient for Liver Transplantation. Hepatology. 41(6): 1-26. June 2005.

Liver transplantation is the most effective treatment for many patients with acute or chronic liver failure resulting from a variety of causes. This article presents the practice guideline from the American Association for the Study of Liver Diseases (AASLD) on the evaluation of patients for liver transplantation. These recommendations are based on a formal review and analysis of the published literature on the topic; several consensus conferences among experts; the American College of Physicians' Manual for Assessing Health Practices and Designing Practice Guidelines; guideline policies produced by professional organizations, including the AASLD and the American Gastroenterological Association; and the authors’ experience in the specified topic. Topics include the indications of liver transplantation, when evaluation for transplantation should be considered, determining the need for liver transplantation, and recipient evaluation at the transplant center. The article offers recommendations for patients with the hepatopulmonary syndrome, portopulmonary hypertension, obesity, cigarette smoking, kidney failure, extrahepatic malignancies, osteoporosis, HIV infection, surgical contraindications, and psychosocial problems. The authors discuss specific indications for liver transplantation, including chronic noncholestatic liver disorders, chronic hepatitis C, chronic hepatitis B, autoimmune hepatitis, alcoholic cirrhosis, cholestatic liver disorders, primary biliary cirrhosis, primary sclerosing cholangitis, childhood cholestatic diseases, metabolic diseases, alpha-1-antitrypsin disease, Wilson disease, nonalcoholic steatohepatitis and cryptogenic cirrhosis, hereditary hemochromatosis, neonatal hemochromatosis, tyrosinemia and glycogen storage disease, metabolic diseases with severe extrahepatic manifestations, amyloidosis and hyperoxaluria, urea cycle and branched-chain amino acid disorders, hepatic malignancies, hepatocellular carcinoma, hepatoblastoma, fibrolamellar hepatocellular carcinoma and hemangioendothelioma, cholangiocarcinoma, and fulminant hepatic failure. The article includes 76 specific recommendations for the evaluation of the patient for liver transplantation. 3 tables. 328 references.

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Primary Biliary Cirrhosis. New England Journal of Medicine. 353(12): 1261-1273. September 22, 2005.

This article reviews advances in primary biliary cirrhosis (PBC), a slowly progressive, autoimmune disease of the liver that primarily affects women. The authors review the typical clinical findings in PBC, pathological findings, natural history and prognosis, causes, environmental factors, autoimmune responses, treatment of symptoms and complications, treatment of underlying disease, and ideas for future research. Common complications of PBC include pruritus, osteoporosis, hyperlipidemia, and portal hypertension. Treatment strategies reviewed include ursodeoxycholic acid (UDCA), colchicines and methotrexate, other drug therapies, and orthotopic liver transplantation. 4 figures. 2 tables. 98 references.

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Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients. Hepatology. 42(5): 1194-1202. November 2005.

This article reports on a study of risk factors and comorbidities in primary biliary cirrhosis (PBC), an autoimmune disease of unknown etiology. The authors included patients with PBC (n =1,032) from 23 referral centers for liver diseases in the United States as well as random-dialed controls (n = 1,041) matched for sex, age, race, and geographical location. Data gathered showed that increased risk of PBC was associated with having a first-degree relative with PBC, a history of urinary tract infections, past smoking, or use of hormone replacement therapies. The frequent use of nail polish slightly increased the risk of having PBC. Other autoimmune diseases, including systemic lupus erythematosus, were found in 32 percent of cases and 13 percent of controls. This study did not confirm other previous reports, such as the increased prevalence of breast cancer in women with PBC. 1 figure. 6 tables. 38 references.

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Autoimmune Liver Disease. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 95-100.

Autoimmune liver disease occurs when the body reacts inappropriately to its own cellular components. Autoimmune liver diseases include autoimmune hepatitis, primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). All three of these diseases can be severe, progressive, and can lead to cirrhosis and death from end-stage liver disease. This chapter on autoimmune liver disease is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the symptoms, etiology, complications, and mechanisms of injury of autoimmune liver disease, then outline recent research advances in the areas of understanding general autoimmunity, causes of autoimmune liver disease, and therapies for autoimmune liver disease. The authors then provide specific research goals in the areas of basic research on pathogenesis, as well as clinical studies of pathogenesis, etiology, and therapy. A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 3 figures. 1 table.

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