Hopp til innhold
NHI.no
Annonse
Informasjon

Primær biliær kolangitt (PBC)

PBC er en autoimmun sykdom av ukjent årsak som særlig rammer kvinner. Tidlig medisinsk behandling bedrer prognosen. Sykdommen ble tidligere kalt primær biliær cirrhose.

Marit kasuistikk kardiologi
PBC er en sjelden sykdom. Det finnes ca. 150-400 med sykdommen per million innbyggere.

Sist oppdatert:

4. jan. 2023

Innhold i artikkelen

Hva er primær biliær kolangitt?

Galleblære, galleveier, bukspyttkjertel

PBC er en autoimmun sykdom hvor immunforsvaret reagerer mot celler i gallegangene i leveren og gir betennelse, arrforandringer og forstyrrelser i leverfunksjonen. Tidlige symptomer er slapphet, plagsom kløe eller tørre slimhinner. Andre symptomer som kommer senere i forløpet kan være fettglinsende avføring, gulsott og blodig oppkast. Symptomer ved langtkommen sykdom er pigmentering og fettknuter i huden.

Annonse

PBC er en sjelden sykdom. Det finnes ca. 150-400 med sykdommen per million innbyggere. Forekomsten varierer rundt om i verden. Den er høyest i Nord-Europa. Cirka 90 prosent av pasientene er kvinner, hyppigst middelaldrende (40-60 år). Tilstanden er sjelden før 30-års alder. Dersom sykdommen påvises tidlig og medisinsk behandling settes i gang da, kan sykdomsutviklingen bremses. 

Vil du vite mer

Dette dokumentet er basert på det profesjonelle dokumentet Primær biliær kolangitt . Referanselisten for dette dokumentet vises nedenfor

  1. Jørgensen KK. Primær biliær cholangitt – sjelden sykdom med nytt navn. Gastroenterologen. Publisert 20.10.2017. Siden besøkt 28.10.2019 gastroenterologen.no
  2. Talwalkar JA, Lindor KD. Primary biliary cirrhosis. Lancet 2003; 362: 53-61. PubMed
  3. 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
  4. Lazaridis KN, Talwalkar JA. Clinical epidemiology of primary biliary cirrhosis: incidence, prevalence, and impact of therapy. J Clin Gastroenterol 2007; 41: 494-500. PubMed
  5. 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
  6. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005; 353: 1261-73. New England Journal of Medicine
  7. Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. N Engl J Med 2008; 357: 1524-9. PubMed
  8. Prieto J, Banales JM, Medina JF. Primary biliary cholangitis: pathogenic mechanisms. Curr Opin Gastroenterol. 2021 Mar 1;37(2):91-98. PMID: 33332913 PubMed
  9. 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
  10. Jones DE. Pathogenesis of primary biliary cirrhosis. J Hepatol 2003; 39: 639-48. PubMed
  11. 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
  12. Jones DEJ, Mackay IR, Snyder N. Primary biliary cirrhosis. BMJ Best Practice, last updated Jan 10, 2017.
  13. Tanaka A. Current understanding of primary biliary cholangitis. Clin Mol Hepatol. 2021 Jan;27(1):1-21. Epub 2020 Dec 3. PMID: 33264835 PubMed
  14. Poupon R. Overview of the management of primary biliary cholangitis. UpToDate, last updated Oct 27, 2021. Siden lest Dec 12, 2022. www.uptodate.com
  15. Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid "mechanisms of action and clinical use in hepatobiliary disorders." J Hepatol 2001; 35: 134-46.
  16. Paumgartner G, Beuers U. Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited. Hepatology 2002; 36: 525-31. PubMed
  17. 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)
  18. 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
  19. 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
  20. 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
  21. 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
  22. Montano-Loza AJ, Wasilenko S, Bintner J, Mason AL. Cyclosporine A protects against primary biliary cirrhosis recurrence after liver transplantation. Am J Transplant. 2010 Apr;10(4):852-858. Epub 2010 Feb 3. PMID: 20132169 PubMed
  23. 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
  24. Poupon R. Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis). UpToDate. Last updated Apr 19, 2022. Site read Dec 20, 2022. www.uptodate.com
  25. 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
  26. 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
Annonse
Annonse