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Primär biliär kolangit

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Definition:
Primär biliär kolangit är en kronisk–progressiv autoimmun kolestatisk sjukdom i de intrahepatiska gallgångarna med inflammation, fibros och till slut cirros med leversvikt och levercancer.
Förekomst:
Incidens 2,1 per en miljon per år, prevalens 285 per miljon invånare. Cirka 80–90 % av patienterna är kvinnor, oftast i medelåldern (40–60 år).
Symtom:
Ibland inga symtom alls. Tidiga tecken är orkeslöshet, besvärande klåda och oförklarlig hyperlipidemi.
Kliniska fynd:
Hepatosplenomegali, hyperpigmentering. Xantomatösa lesioner förekommer. Ikterus och andra tecken på avancerad leversjukdom i ett senare förlopp. 
Diagnos:
Kräver två av följande kriterier: förhöjda alkaliska fosfataser (ALP) under mer än sex månader, förhöjda antikroppar och/eller leverbiopsi med bild som vid primär biliär kolangit.
Behandling:
Ursodeoxicholsyra (UDCA) som förstahands behandling. För UDCA non-responderskan behandling med obeticholsyra (OCA) övervägas. Symptomatisk behandling av klåda och osteoporos.

 

  1. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005; 353: 1261-73. New England Journal of Medicine  
  2. Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology 2009;50:291-308. PMID: 19554543 PubMed  
  3. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol 2012;56:1181-8. PMID: 22245904 PubMed  
  4. S Wahlin, JF Ludvigsson, F Soderdahl, M et al. Incidence, Prevalence and Outcome of Primary Biliary Cholangitis in Sweden HEPATOLOGY 66, 175A-176A
  5. Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. N Engl J Med 2008; 357: 1524-9. PubMed  
  6. Hirschfield GM, Gershwin ME. The immunobiology and pathophysiology of primary biliary cirrhosis. Annu Rev Pathol 2013;8:303-30. PMID: 23347352 PubMed  
  7. Liaskou E, Hirschfield GM, Gershwin ME. Mechanisms of tissue injury in autoimmune liver diseases. Semin Immunopathol 2014;36:553-68. PMID: 25082647 PubMed  
  8. Hirschfield GM, Liu X, Xu C, et al. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N Engl J Med 2009;360:2544-55. PMID: 19458352 PubMed  
  9. Cordell HJ, Han Y, Mells GF, et al. International genome-wide meta-analysis identifies new primary biliary cirrhosis risk loci and targetable pathogenic pathways. Nat Commun 2015;6:8019. PMID: 26394269 PubMed  
  10. Webb GJ, Hirschfield GM. Using GWAS to identify genetic predisposition in hepatic autoimmunity. J Autoimmun 2016;66:25-39. PMID: 26347073 PubMed  
  11. European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017 Jul;67(1):145-172. doi: 10.1016/j.jhep.2017.03.022. Epub 2017 Apr 18. PMID: 28427765 PubMed  
  12. Dahlqvist G, Gaouar F, Carrat F et al. Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis. Hepatology. 2017 Jan;65(1):152-163. PMID: 27688145 PubMed  
  13. Corpechot C, Carrat F, Poujol-Robert A et al. Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis. Hepatology. 2012 Jul;56(1):198-208. PMID 22271046
  14. Beuers U, Trauner M, Jansen P, Poupon R. New paradigms in the treatment of hepatic cholestasis: from UDCA to FXR, PXR and beyond. J Hepatol. 2015 Apr;62(1 Suppl):S25-37. PMID: 25920087 PubMed  
  15. Paumgartner G, Beuers U. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 2002; 36: 525-31. PubMed  
  16. Nevens F, Andreone P, Mazzella G, et al. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis. N Engl J Med 2016;375:631-43. PMID: 27532829 PubMed  
  17. Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet 2015 ; Oct 17;386(10003): 1565-75. pmid:26364546 PubMed  
  18. Prince MI, Burt AD, Jones DE. Hepatitis and liver dysfunction with rifampicin therapy for pruritus in primary biliary cirrhosis. Gut 2002; 50: 436-39. Gut  
  19. Ritzel U, Leonhardt U, Näther M, Schäfer G, Armstrong WW, Ramadori G. Simvastatin in primary biliary cirrhosis: effects on serum lipids and distinct disease markers. J Hepatol 2002; 36: 454-8. PubMed  
  20. Zein CO, Jorgensen RA, Clarke B, et al. Alendronate improves bone mineral density in primary biliary cirrhosis: a randomized placebo-controlled trial. Hepatology 2005; 42: 762-71. PubMed  
  21. Fosby B, Melum E, Bjøro K et al. Liver transplantation in the Nordic countries - An intention to treat and post-transplant analysis from The Nordic Liver Transplant Registry 1982-2013. Scand J Gastroenterol. 2015 Jun;50(6):797-808. PMID: 25959101 PubMed  
  22. Bosch A, Dumortier J, Maucort-Boulch D et al. Preventive administration of UDCA after liver transplantation for primary biliary cirrhosis is associated with a lower risk of disease recurrence. J Hepatol. 2015 Dec;63(6):1449-58. PMID: 26282232 PubMed  
  23. Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006;130:715-20. PMID: 16530513 PubMed  
  24. Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008;48:871-7. PMID: 18752324 PubMed  
  25. Kuiper EM, Hansen BE, de Vries RA, et al. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009;136:1281-7. PMID: 19208346 PubMed  
  26. Kumagi T, Guindi M, Fischer SE, et al. Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis. Am J Gastroenterol 2010;105:2186-94. PMID: 20502446 PubMed  
  27. Corpechot C, Chazouilleres O, Poupon R. Early primary biliary cirrhosis: biochemical response to treatment and prediction of long-term outcome. J Hepatol 2011;55:1361-7. PMID: 21703194 PubMed  
  28. Lammers WJ, Hirschfield GM, Corpechot C, et al. Development and Validation of a Scoring System to Predict Outcomes of Patients With Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy. Gastroenterology 2015;149:1804-12 e4. PMID: 26261009 PubMed  
  29. Carbone M, Sharp SJ, Flack S, et al. The UK-PBC risk scores: Derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatology 2016;63:930-50. PMID: 26223498 PubMed  
  30. Liang Y, Yang Z, Zhong R. Primary biliary cirrhosis and cancer risk: a systematic review and meta-analysis. Hepatology 2012;56:1409-17. PMID: 22504852 PubMed  
  31. Longo M, Crosignani A, Battezzati M, et al. Hyperlipidemic state and cardiovascular risk in primary biliary cirrhosis. Gut 2002; 51: 265-69. Gut  
  • Hanns-Ulrich Marschall, professor och universitetssjukhusöverläkare, Medicinkliniken, Sahlgrenska Universitetssjukhuset, Göteborg
  • Anna Nager, med dr och specialist i allmänmedicin, Karolinska institutet