Zusammenfassung
Die Diagnose der primär biliären Zirrhose (PBC), einer immunvermittelten Entzündung der kleinen intrahepatischen Gallenwege, die im Spätstadium zu einer biliären Zirrhose fortschreitet, beruht auf charakteristischen klinischen, histopathologischen und laborchemischen Befunden (Erhöhung von γGT und alkalischer Phosphatase im Serum; Nachweis antimitochondrialer Antikörper, AMA). Evidenzbasierte Behandlung der PBC beeinflusst den Krankheitsverlauf günstig und verbessert die Langzeitprognose betroffener Patienten. Die frühe Diagnose und medikamentöse Therapie mit Ursodeoxycholsäure sind von entscheidender Bedeutung, um ein Fortschreiten zur Leberzirrhose und eine Dekompensation der fortgeschrittenen Lebererkrankung möglicherweise zu verhindern. Weitere medikamentöse Therapieoptionen sind derzeit in Evaluation. Die Lebertransplantation stellt eine effektive Behandlung für das Endstadium der PBC dar.
Abstract
Primary biliary cirrhosis (PBC) is characterized by immune-mediated destruction of small intrahepatic bile ducts and subsequent development of liver fibrosis and cirrhosis, occuring mainly in middle-aged women. PBC is diagnosed on the basis of a cholestatic serum enzyme pattern, elevated serum IgM, the presence of antimitochondrial antibodies (AMA) in serum directed against the E2 subunit of the pyruvate dehydrogenase complex, and a „florid bile duct lesion” of mid size intrahepatic bile ducts and bile duct paucity. The cholestatic serum enzyme pattern and serum AMA are mandatory for the diagnosis. PBC is frequently associated with other autoimmune disorders such as Sjögren’s syndrome, Hashimoto’s thyroiditis, and celiac disease. Early diagnosis and medical therapy with ursodeoxycholic acid aim to hinder progression of the disease toward cirrhosis. Additional medical treatments are under evaluation. Liver transplantation is an effective treatment of end-stage PBC.
This is a preview of subscription content, log in via an institution to check access.
Literatur
Gershwin ME, Mackay IR (2008) The causes of primary biliary cirrhosis: Convenient and inconvenient truths. Hepatology 47: 737–745
Invernizzi P, Selmi C, Mackay IR et al (2005) From bases to basis: linking genetics to causation in primary biliary cirrhosis. Clin Gastroenterol Hepatol 3: 401–410
Invernizzi P, Miozzo M, Battezzati PM et al (20047) Frequency of monosomy X in women with primary biliary cirrhosis. Lancet 363: 533–535
Rieger R, Leung PS, Jeddeloh MR et al (2006) Identification of 2-nonynoic acid, a cosmetic component, as a potential trigger of primary biliary cirrhosis. J Autoimmun 27: 7–16
Ala A, Stanca CM, Bu-Ghanim M et al (2006) Increased prevalence of primary biliary cirrhosis near Superfund toxic waste sites. Hepatology 43: 525–531
Selmi C, Balkwill DL, Invernizzi P et al (2003) Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium. Hepatology 38: 1250–1257
Bogdanos DP, Baum H, Okamoto M et al (2005) Primary biliary cirrhosis is characterized by IgG3 antibodies cross-reactive with the major mitochondrial autoepitope and its Lactobacillus mimic. Hepatology 42: 458–465
Bogdanos D, Pusl T, Rust C et al (2008) Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis. J Hepatol 49: 466–473
Newton JL, Gibson GJ, Tomlinson M et al (2006) Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology 44: 91–98
Jones DE, Newton JL (2007) An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis. Aliment Pharmacol Ther 25: 471–476
Kremer AE, Beuers U, Oude-Elferink RP, Pusl T (2008) Pathogenesis and treatment of pruritus in cholestasis. Drugs 68: 2163–2182
Corpechot C, Carrat F, Bahr A et al (2005) The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 128: 297–303
Corpechot C, Abenavoli L, Rabahi N et al (2008) Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 48: 871–877
Pares A, Caballeria L, Rodes J (2006) Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 130: 715–720
Beuers U (2006) Drug insight: Mechanisms and sites of action of ursodeoxycholic acid in cholestasis. Nat Clin Pract 3: 318–328
Combes B, Emerson SS, Flye NL et al (2005) Methotrexate (MTX) plus ursodeoxycholic acid (UDCA) in the treatment of primary biliary cirrhosis. Hepatology 42: 1184–1193
Hempfling W, Grunhage F, Dilger K et al (2003) Pharmacokinetics and pharmacodynamic action of budesonide in early- and late-stage primary biliary cirrhosis. Hepatology 38: 196–202
Leuschner M, Maier KP, Schlichting J et al (1999) Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial. Gastroenterology 117: 918–925
Rautiainen H, Karkkainen P, Karvonen AL et al (2005) Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial. Hepatology 41: 747–752
Zollner G, Marschall HU, Wagner M, Trauner M (2006) Role of nuclear receptors in the adaptive response to bile acids and cholestasis: pathogenetic and therapeutic considerations. Mol Pharm 3: 231–251
Interessenkonflikt
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Beuers, U., Hohenester, S. Primär biliäre Zirrhose. Gastroenterologe 4, 110–114 (2009). https://doi.org/10.1007/s11377-008-0258-y
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11377-008-0258-y