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Primær biliær kolangitt

Sist revidert:
Sist revidert av:
Definisjon:
En autoimmun, kronisk, kolestatisk sykdom i de intrahepatiske galleganger med inflammasjon, fibrose og til slutt leversvikt. Sykdommen ble tidligere kalt primær biliær cirrhose
Forekomst:
Sjelden. Prevalens 150-400 per million
Symptomer:
Tidlige tegn er slapphet, plagsom kløe og uforklarlig hyperlipidemi
Funn:
Hepatosplenomegali, hyperpigmentering, xantomatøse lesjoner kan forekomme i hud, sener eller rundt øyebryn. Ikterus eventuelt, men er et sent innsettende tegn
Diagnostikk:
Biokjemiske prøver viser kolestatisk mønster. Anti-mitokondrie-antistoffer har høy sensitivitet og spesifisitet
Behandling:
Primært symptomatisk. Ursodeoksykolsyre brukes mye, men har usikker effekt
  1. Jones DEJ, Mackay IR, Snyder N. Primary biliary cirrhosis. BMJ Best Practice, last updated Jan 10, 2017.
  2. Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet 2003; 362: 53-61. PubMed  
  3. Jørgensen KK. Primær biliær cholangitt – sjelden sykdom med nytt navn. Gastroenterologen. Publisert 20.10.2017. Siden besøkt 28.10.2019 gastroenterologen.no  
  4. Prince MI, Chetwynd A, Diggle P, Jarner M, Metcalf JV, James OF. The geographical distribution of primary biliary cirrhosis in a well-defined cohort. Hepatology 2001; 34: 1083-88. PubMed  
  5. Lazaridis KN, Talwalkar JA. Clinical epidemiology of primary biliary cirrhosis: incidence, prevalence, and impact of therapy. J Clin Gastroenterol 2007; 41: 494-500. PubMed  
  6. Kim WR, Lindor KD, Locke GR III, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology 2000; 119: 1631-6. Gastroenterology  
  7. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005; 353: 1261-73. New England Journal of Medicine  
  8. Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. N Engl J Med 2008; 357: 1524-9. PubMed  
  9. He XS, Ansari AA, Ridgway WM, Coppel RL, Gershwin ME. New insights to the immunopathology and autoimmune responses in primary biliary cirrhosis. Cell Immunol 2006; 239: 1-13. PubMed  
  10. Selmi C, Balkwill DL, Invernizzi P, et al. Patients with primary biliary cirrhosis react against a ubiquitous xenobiotic-metabolizing bacterium. Hepatology 2003; 38: 1250-7. PubMed  
  11. Ueno Y, Moritoki Y, Shimosegawa T, Gershwin ME. Primary biliary cirrhosis: what we know and what we want to know about human PBC and spontaneous PBC mouse models. J Gastroenterol 2007; 42: 189-95. PubMed  
  12. Jones DE. Pathogenesis of primary biliary cirrhosis. J Hepatol 2003; 39: 639-48. PubMed  
  13. Howel D, Fischbacher CM, Bhopal RS, Gray J, Metcalf JV, James OF. An exploratory population-based case-control study of primary biliary cirrhosis. Hepatology 2000; 31: 1055-60. PubMed  
  14. Zein CO, Angulo P, Lindor KD. When is a liver biopsy needed in the diagnosis of primary biliary cirrhosis? Clin Gastroenterol Hepatol 2003; 1: 89-96. PubMed  
  15. Rudic JS, Poropat G, Krstic MN et al. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database of Systematic Reviews, Dec 2012, CD000551.pub3. Cochrane (DOI)  
  16. Ozaslan E, Efe C, Heurgué-Berlot A3, et al. Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis. Clin Gastroenterol Hepatol 2014 doi:10.1016/j.cgh.2013.09.021 DOI  
  17. Poupon R. Overview of the treatment of primary biliary cholangitis. UpToDate, last updated Mar 04, 2019. UpToDate  
  18. Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid "mechanisms of action and clinical use in hepatobiliary disorders." J Hepatol 2001; 35: 134-46.
  19. Paumgartner G, Beuers U. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 2002; 36: 525-31. PubMed  
  20. Shi J, Wu C, Lin Y, Chen YX, Zhu L, Xie WF. Long-term effects of mid-dose ursodeoxycholic acid in primary biliary cirrhosis: a meta-analysis of randomized controlled trials. Am J Gastroenterol 2006; 101: 1529-38. PubMed  
  21. Siegel JL, Jorgensen R, Angulo P, Lindor KD. Treatment with ursodeoxycholic acid is associated with weight gain in patients with primary biliary cirrhosis. J Clin Gastroenterol 2003; 37: 183-5. PubMed  
  22. 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  
  23. 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  
  24. 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  
  25. Liermann Garcia RF, Evangelista Garcia C, McMaster P, Neuberger J. Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology 2001; 33: 22-27. PubMed  
  26. ter Borg PCJ, Schalm SW, Hansen B, van Buuren HR. Prognosis of ursodeoxycholic acid-treated patients with primary biliary cirrhosis: results of a 10-yr cohort study involving 297 patients. Am J Gastroenterol 2006; 101: 2044-50. PubMed  
  27. Corpechot C, Carrat F, Bahr A, Chrétein Y, Poupon RE, Poupon R. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005; 128: 297-303. Gastroenterology  
  • Terje Johannessen, professor i allmennmedisin, Trondheim (tilpasning til NEL)

Tidligere fagmedarbeidere

  • Eiliv Brenna, overlege, Medisinsk avdeling, Regionsykehuset i Trondheim
  • Svein Ødegaard, overlege og professor, Haukeland sykehus og Nasjonalt senter for gastroenterologisk ultrasonografi, Bergen