Primær skleroserende kolangitt
Sist revidert:
Sist revidert av:Terje Johannessen
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
- Kowdley KV. Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis. UpToDate, last updated May 21, 2018. UpToDate
- Gan SI. Primary sclerosing cholangitis. BestPractice, last updated Feb 17, 2017. bestpractice.bmj.com
- 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
- 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
- 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
- Chapman R, Cullen S. Etiopathogenesis of primary sclerosing cholangitis. World J Gastroenterol 2008; 14: 3350-9. PubMed
- Lindor KD, Kowdley KV, Harrison ME, American College of Gastroenterology. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol 2015; 110:646. PubMed
- Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51: 660-78. PubMed
- Angulo P, Peter JB, Gershwin ME, et al. Serum autoantibodies in patients with primary sclerosing cholangitis. J Hepatol 2000; 32:182. PubMed
- Said K, Glaumann H, Bergquist A. Gallbladder disease in patients with primary sclerosing cholangitis. J Hepatol 2008; 48:598. PubMed
- Kowdley KV. Primary sclerosing cholangitis in adults: Management. UpToDate, last updated Nov 25, 2019. UpToDate
- 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
- 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
- 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.
- 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