Hopp til hovedinnhold

Primær skleroserende kolangitt (PSC)

Sist revidert:
Sist revidert av:


Definisjon:
En kronisk fibroserende betennelse av ukjent årsak i middels store og store intra- og ekstrahepatiske galleganger, først og fremst hos pasienter med ulcerøs kolitt, som til slutt kan føre til leversvikt
Forekomst:
Sjelden tilstand. Forekommer særlig blant unge menn
Symptomer:
Få symptomer tidlig i forløpet. Senere tiltakende slapphet, hudkløe og ikterus
Funn:
Få tidlige funn, men økende tegn til leversvikt ettersom tilstanden progredierer
Diagnostikk:
Stigende ALP er den viktigste markøren. Kolestatisk mønster. Ulike antistoffer kan påvises. Retrograd kolangiografi kan vise karakteristiske forandringer i galleveiene
Behandling:
Det finnes ingen optimal behandling verken medikamentelt eller endoskopisk. Levertransplantasjon kan bli aktuelt
  1. Kowdley KV. Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis. UpToDate, last updated May 21, 2018. UpToDate  
  2. Gan SI. Primary sclerosing cholangitis. BestPractice, last updated Feb 17, 2017. bestpractice.bmj.com  
  3. Bergquist A, Said K, Broome U. Changes over a 20-year period in the clinical presentation of primary sclerosing cholangitis in Sweden. Scand J Gastroenterol 2007;42:88-93. PubMed  
  4. Boberg KM, Aadland E, Jahnsen J, et al. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998;33:99-103. PubMed  
  5. Bergquist A, Montgomery SM, Bahmanyar S, et al. Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2008;6:939-943. PubMed  
  6. Chapman R, Cullen S. Etiopathogenesis of primary sclerosing cholangitis. World J Gastroenterol 2008; 14: 3350-9. PubMed  
  7. Lindor KD, Kowdley KV, Harrison ME, American College of Gastroenterology. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol 2015; 110:646. PubMed  
  8. Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51: 660-78. PubMed  
  9. Angulo P, Peter JB, Gershwin ME, et al. Serum autoantibodies in patients with primary sclerosing cholangitis. J Hepatol 2000; 32:182. PubMed  
  10. Said K, Glaumann H, Bergquist A. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 2008; 48:598. PubMed  
  11. Kowdley KV. Primary sclerosing cholangitis in adults: Management. UpToDate, last updated Nov 25, 2019. UpToDate  
  12. Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009; 50: 808-14. PubMed  
  13. Tischendorf JJ, Hecker H, Krüger M, et al. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study. Am J Gastroenterol 2007; 102:107. PubMed  
  14. Navaneethan U, Venkatesh PG, Lashner BA, et al. The Impact of ulcerative colitis on the long-term outcome of patients with primary sclerosing cholangitis. Aliment Pharmacol Ther 2012 March.
  15. Ngu JH, Gearry RB, Wright AJ, Stedman CA. Inflammatory bowel disease is associated with poor outcomes of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol 2011; 9:1092. PubMed  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Hermod Petersen, professor og overlege, Norges teknisk-naturvitenskapelige universitet og Medisinsk avdeling, Regionsykehuset i Trondheim
  • Eiliv Brenna, overlege, Medisinsk avdeling, Regionsykehuset i Trondheim