Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy
Open Access
- 1 January 2015
- journal article
- review article
- Published by Baishideng Publishing Group Inc. in World Journal of Hepatology
- Vol. 7 (7), 926-941
- https://doi.org/10.4254/wjh.v7.i7.926
Abstract
Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren's syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, cholinergic agents, and scrupulous oral and dental care. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.Keywords
This publication has 158 references indexed in Scilit:
- Approach to a Patient with Elevated Serum Alkaline PhosphataseClinics in Liver Disease, 2012
- 964 THE FARNESOID X RECEPTOR (FXR) AGONIST OBETICHOLIC ACID (OCA) INCREASES PLASMA FGF-19 CONCENTRATIONS AND DECREASES BILE ACID SYNTHESIS IN PRIMARY BILIARY CIRRHOSIS (PBC)Journal of Hepatology, 2012
- Management of hepatocellular carcinoma: An updateJournal of Hepatology, 2011
- Primary biliary cirrhosisBest Practice & Research Clinical Gastroenterology, 2010
- Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosisJournal of Hepatology, 2010
- Surveillance for hepatocellular carcinoma in patients with primary biliary cirrhosisJournal of Hepatology, 2008
- Incidence of cancer in primary biliary cirrhosis: The mayo experienceJournal of Hepatology, 1999
- BudesonideDrugs, 1995
- Oral glucocorticoids and their complications: A reviewJournal of the American Academy of Dermatology, 1986
- Portal hypertension in primary biliary cirrhosisGut, 1971