The diagnosis of primary biliary cirrhosis |
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| Affiliation: | 1. Department of Medicine, McGill University, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, H3T 1E3 Quebec, Canada;2. Division of Rheumatology, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, H3T 1E3 Quebec, Canada;3. Lady Davis Institute, Jewish General Hospital, 3755 Côte Ste-Catherine, Montréal, Quebec H3T 1E3, Canada;4. Faculty of Medicine, University of Calgary, 3330 Hospital Drive, Calgary, T2N 4N1 Alberta, Canada;1. Department of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands;2. NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, England;3. Liver Clinic, Toronto Western & General Hospital, University Health Network, Toronto, Ontario, Canada;4. Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano, Italy;5. Divison of Gastroenterology and Hepatology, University of Alberta, Edmonton, Alberta, Canada;6. Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands;7. Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy;8. Centre de Référence des Maladies Inflammatoires des VoiesBiliaires, Hôpital Saint-Antoine, APHP, Paris, France;9. Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, Texas;10. Department of Health Sciences, Università degli Studi di Milano, Milan, Italy;11. Liver Unit, Hospital Clínic, CIBERehd, IDIBAPS, University of Barcelona, Barcelona, Spain;12. Department of Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium;13. The Sheila Sherlock Liver Centre, The Royal Free Hospital, London, England;14. Liver Center of Excellence, Digestive Disease Institute, Virginia Mason Medical Center, Seattle, Washington;15. Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, Japan;16. Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota;17. College of Health Solutions, Arizona State University, Phoenix, Arizona;1. National Institute for Health Research (NIHR) Birmingham Liver Biomedical Research Unit (BRU) and Centre for Liver Research, University of Birmingham, Birmingham, UK;2. Liver Unit, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK;3. Department of Internal Medicine IV, Jena University Hospital, Friedrich Schiller University Jena, Jena, Germany;4. Center for Sepsis Control and Care (CSCC), Jena University Hospital, Friedrich Schiller University Jena, Germany;5. Toronto Center for Liver Diseases, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada;6. Gastroenterology and Metabology, Ehime University, Graduate School of Medicine, Shitsukawa To-on, Ehime, Japan;7. Royal Hospital, Sultanate of Oman, Oman;8. Organ Donation and Transplant, NHS Blood and Transplant, Bristol, UK;9. Division of Gastroenterology & Hepatology, Erasmus MC University Hospital, Rotterdam, The Netherlands;1. Department of Paediatrics, Wilhelmina Children''s Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands;2. Department of Paediatrics, Emma Children''s Hospital, Academic Medical Centre, Amsterdam, The Netherlands;3. Julius Centre for Health Sciences and Primary Care, University Medical Centre Utrecht, Utrecht, The Netherlands;4. Expert Centre for Chronic Fatigue, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands;1. Department of Anesthesiology, University of Utah Health Sciences Center, Salt Lake City, UT, USA;2. Department of Medicine, University of Utah Health Sciences Center, Salt Lake City, UT, USA;3. Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA;4. Neuroscience Program, University of Utah Health Sciences Center, Salt Lake City, UT, USA;5. Department of Exercise and Sport Science, University of Utah, Salt Lake City, UT, USA;6. Fatigue Consultation Clinic, Salt Lake City, UT, USA;1. Department of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands;2. NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, United Kingdom;3. Centre de Référence des Maladies Inflammatoires des Voies Biliaires, Hôpital Saint-Antoine, APHP, Paris, France;4. Department of Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium;5. Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota;6. Arizona State University, College of Health Solutions, Phoenix, Arizona;7. Liver Clinic, Toronto Western and General Hospital, University Health Network, Toronto, Ontario, Canada;8. Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy;9. Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands;10. Digestive and Liver Diseases, UT Southwestern Medical Center, Dallas, Texas;11. Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano, Italy;12. Department of Health Sciences, Università degli Studi di Milano, Milan, Italy;13. Liver Unit, Hospital Clínic, CIBERehd, IDIBAPS, University of Barcelona, Barcelona, Spain;14. The Sheila Sherlock Liver Centre, The Royal Free Hospital, London, United Kingdom;15. Divison of Gastroenterology and Hepatology, University of Alberta, Edmonton, Alberta, Canada;16. Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, Washington;17. Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, Japan |
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| Abstract: | Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by the immune mediated destruction of small intrahepatic bile duct epithelial cells leading to cholestasis and cirrhosis. The autoimmune basis of PBC is supported by the highly specific anti-mitochondrial antibodies (AMAs) and autoreactive T cells, the former being the basis for diagnosis in the vast majority of cases. Although a rare disease, the incidence rates of PBC have been increasing, possibly due to increased testing and diagnosis as opposed to a true increase in disease incidence. Presently, most cases are asymptomatic and only suspected based upon routine liver tests. Those with symptoms typically complain of pruritus and fatigue. The diagnosis of PBC is based on the presence of at least 2 of 3 key criteria including a persistently elevated serum alkaline phosphatase, the presence of serum AMAs, and liver histology consistent with PBC. Anti-nuclear antibodies specific to PBC are useful in cases in which AMAs are not detected and may indicate a more aggressive course. Ursodeoxycholic acid is the only proven therapy for PBC and in most cases can delay or prevent disease progression. However, a subgroup of patients does not adequately respond to ursodeoxycholic acid and for whom new therapies are needed. |
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