Primary Sclerosing Cholangitis As an Intractable Disease

Overview

Journal Intractable Rare Dis Res

Date 2014 Oct 25

PMID 25343066

Citations 1

Authors

Sumihito Tamura

Yasuhiko Sugawara

Norihiro Kokudo

Affiliations

Soon will be listed here.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin which eventually results in liver cirrhosis. The disease is reported to be more common among the Western population than in the Asian population. Asian experience remains limited. Diagnosis and treatment standards in the Far East have largely followed those in the West, including liver transplantation. Unlike in the West, however, recent reports from Japan have presented with a higher recurrence rate of PSC after liver transplantation, suggesting the intractable nature of the disease even after the replacement of the entire affected organ.

Citing Articles

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis.

Bajer L, Kverka M, Kostovcik M, Macinga P, Dvorak J, Stehlikova Z World J Gastroenterol. 2017; 23(25):4548-4558.

PMID: 28740343 PMC: 5504370. DOI: 10.3748/wjg.v23.i25.4548.

References

Brandsaeter B, Schrumpf E, Bentdal O, Brabrand K, Smith H, Abildgaard A . Recurrent primary sclerosing cholangitis after liver transplantation: a magnetic resonance cholangiography study with analyses of predictive factors. Liver Transpl. 2005; 11(11):1361-9. DOI: 10.1002/lt.20444. View

Soejima Y, Harada N, Shimada M, Suehiro T, Minagawa R, Hiroshige S . Perioperative management and complications in donors related to living-donor liver transplantation. Surgery. 2002; 131(1 Suppl):S195-9. DOI: 10.1067/msy.2002.119576. View

Oya H, Sato Y, Yamamoto S, Takeishi T, Kobayashi T, Hatakeyama K . Living related donor liver transplantation for primary sclerosing cholangitis with hepatocellular carcinoma and Crohn's disease: a case report. Transplant Proc. 2004; 36(8):2297-8. DOI: 10.1016/j.transproceed.2004.07.041. View

Khettry U, Keaveny A, Goldar-Najafi A, Lewis W, Pomfret E, Pomposelli J . Liver transplantation for primary sclerosing cholangitis: a long-term clinicopathologic study. Hum Pathol. 2003; 34(11):1127-36. DOI: 10.1053/j.humpath.2003.07.015. View

Sugawara Y, Makuuchi M . Technical advances in living-related liver transplantation. J Hepatobiliary Pancreat Surg. 1999; 6(3):245-53. DOI: 10.1007/s005340050114. View

Sheng R, CAMPBELL W, Zajko A, BARON R . Cholangiographic features of biliary strictures after liver transplantation for primary sclerosing cholangitis: evidence of recurrent disease. AJR Am J Roentgenol. 1996; 166(5):1109-13. DOI: 10.2214/ajr.166.5.8615253. View

Vera A, Moledina S, Gunson B, Hubscher S, Mirza D, Olliff S . Risk factors for recurrence of primary sclerosing cholangitis of liver allograft. Lancet. 2002; 360(9349):1943-4. DOI: 10.1016/S0140-6736(02)11861-7. View

Tamura S, Sugawara Y, Kokudo N . Donor evaluation and hepatectomy for living-donor liver transplantation. J Hepatobiliary Pancreat Surg. 2008; 15(2):79-91. DOI: 10.1007/s00534-007-1294-6. View

Portincasa P, Vacca M, Moschetta A, Petruzzelli M, Palasciano G, van Erpecum K . Primary sclerosing cholangitis: updates in diagnosis and therapy. World J Gastroenterol. 2004; 11(1):7-16. PMC: 4205387. DOI: 10.3748/wjg.v11.i1.7. View

10.

Heimbach J, Menon K, Ishitani M, Nyberg S, Jankowski C, Lindor K . Living donor liver transplantation using a right lobe graft in an adult with situs inversus. Liver Transpl. 2005; 11(1):111-3. DOI: 10.1002/lt.20313. View

11.

Nakamura M, Fuchinoue S, Nakajima I, Kitajima K, Tojimbara T, Takasaki K . Three cases of sequential liver-kidney transplantation from living-related donors. Nephrol Dial Transplant. 2001; 16(1):166-8. DOI: 10.1093/ndt/16.1.166. View

12.

Tanaka A, Takamori Y, Toda G, Ohnishi S, Takikawa H . Outcome and prognostic factors of 391 Japanese patients with primary sclerosing cholangitis. Liver Int. 2008; 28(7):983-9. DOI: 10.1111/j.1478-3231.2008.01726.x. View

13.

Kugelmas M, Spiegelman P, Osgood M, Young D, Trotter J, Steinberg T . Different immunosuppressive regimens and recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2003; 9(7):727-32. DOI: 10.1053/jlts.2003.50143. View

14.

Hayashi P, Forman L, Steinberg T, Bak T, Wachs M, Kugelmas M . Model for End-Stage Liver Disease score does not predict patient or graft survival in living donor liver transplant recipients. Liver Transpl. 2003; 9(7):737-40. DOI: 10.1053/jlts.2003.50122. View

15.

Alabraba E, Nightingale P, Gunson B, Hubscher S, Olliff S, Mirza D . A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl. 2009; 15(3):330-40. DOI: 10.1002/lt.21679. View

16.

Wachs M, Bak T, Karrer F, Everson G, Shrestha R, Trouillot T . Adult living donor liver transplantation using a right hepatic lobe. Transplantation. 1998; 66(10):1313-6. DOI: 10.1097/00007890-199811270-00008. View

17.

Chapman R, Arborgh B, Rhodes J, Summerfield J, Dick R, Scheuer P . Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut. 1980; 21(10):870-7. PMC: 1419383. DOI: 10.1136/gut.21.10.870. View

18.

MacFaul G, Chapman R . Sclerosing cholangitis. Curr Opin Gastroenterol. 2005; 21(3):348-53. DOI: 10.1097/01.mog.0000155359.43763.cc. View

19.

Yamagiwa S, Ichida T . Recurrence of primary biliary cirrhosis and primary sclerosing cholangitis after liver transplantation in Japan. Hepatol Res. 2007; 37 Suppl 3:S449-54. DOI: 10.1111/j.1872-034X.2007.00250.x. View

20.

Harrison R, Davies M, Neuberger J, Hubscher S . Fibrous and obliterative cholangitis in liver allografts: evidence of recurrent primary sclerosing cholangitis?. Hepatology. 1994; 20(2):356-61. View