Primary sclerosing cholangitis.drquiyum
- 1. Primary sclerosing cholangitis Dr. Mostafa MamoonWarid Phase- B, Hepatobilary Surgery Bangabandhu Sheikh Mujib Medical University
- 2. Introduction ▪ Primary sclerosing cholangitis (PSC) is a chronic, idiopathic, cholestatic liver disease characterized histologically by peribiliary inflammation and fibrosis. ▪ It can lead to end stage cirrhosis and is a recognized risk factor for hepatobiliary cancers ▪ This carries a median liver transplant (LT)-free survival time of approximately 15 years (Jussila et al, 2013; Karlsen et al, 2010b; Weismuller et al, 2008). ▪ Due to its progressive nature and lack of effective pharmacotherapy, and despite being a relatively rare disorder, PSC is the fifth most common indication for LT (UNOS, 2015; Bjoro et al, 2006; Karlsen et al, 2010)
- 3. Epidemiology ▪ PSC most commonly affects males. ▪ It commonly affects during the fourth decade of life (Angulo et al, 1999). ▪ Strong association with inflammatory bowel disease (IBD)
- 5. Clinical features ▪ Right upper quadrant discomfort ▪ Jaundice ▪ Pruritus ▪ Fever ▪ Weight loss
- 6. Diagnosis ▪ Clinical features with ▪ (1) a chronically cholestatic serum biochemical profile ▪ (2) cholangiography demonstrating multifocal intrahepatic and/or extrahepatic biliary strictures and segmental dilations ▪ (3) compatible features (e.g., chronic cholangitis, ductular proliferation, and periductal fibrosis) on liver biopsy
- 10. Classification
- 11. Staging of PSC ▪ Histopathology from liver tissue ▪ According to Ludwig et al. (1986)
- 12. Treatment ▪ Medical management ▪ Surgical management
- 13. Medical management ▪ Ursodeoxycholic acid (UDCA) ▪ Antibiotics ▪ Vitamin K ▪ Cholestyramines ▪ Steroids
- 14. Ursodeoxycholic acid (UDCA) ▪ Low dose – 10-15mg/kg/day – Significant biochemical improvement – No difference in formation of Cholangiocarcinoma (CCA) or death ▪ High dose – 28-30mg/kg/day – 2 fold increase in adverse effect due to toxic metabolites
- 15. Ursodeoxycholic acid (UDCA) ▪ Intermediate dose – 17-23mg/kg/day – Improves biochemical profile significantly – Relative reduction in formation of CCA – Less toxic metabolites than high dose.
- 16. Surgical management ▪ ERCP – For management of “dominant stricture” Dominant stricture is loosely defined as a stenosis with a diameter of less than or equal to 1.5 mm in the common bile duct or less than or equal to 1 mm in the hepatic duct – Brush cytology for cholangiocarcinoma surveillance
- 17. ▪ Resection of Cholangiocarcinoma (CCA) with PSC – When to do ▪ Resectable ▪ Don’t have cirrhotic stage PSC ▪ Not suitable for liver transplantation (LT) – Precaution ▪ CCA is often mulifocal ▪ Hepatic fibrosis/ low hepatic reserve may preclude safe resection ▪ Recurrent disease/death occurs in 90% patient
- 18. Orthotopic liver transplant ▪ PSC IS 5th most common cause of LT ▪ 1 year survival 90% ▪ 5 years survival 80% ▪ Roux-en-Y Bilioenteric anastomosis is superior technique (Welsh et al, 2004) ▪ Post-LT recurrent PSC occur in up to 34% of deceased donor LTs (Vera et al,2002) and 67% of living-related donor LTs (Egawa et al, 2009; Tamura et al, 2007)
- 19. ▪ Median survival without redo-LT for these patients is estimated to be approximately 4 years (Campsen et al, 2008) ▪ PSC with CCA needs neoadjuvant radiosensitizing chemotherapy, external beam radiotherapy, and ERC-delivered transluminal brachytherapy, followed by oral capecitabine until staging laparotomy or laparoscopy immediately prior to LT to re-verify candidacy (DeVreede et al, 2000; Rea et al, 2005).
- 20. Conclusion ▪ PSC is a chronic, cholestatic, premalignant cholangiopathy of unknown etiology characterized by fibrous obliteration of the intrahepatic and/or extrahepatic biliary tree and a strong association with IBD ▪ Effective medical therapy has yet to be established ▪ LT is an excellent option in carefully selected patients with PSC associated liver failure or hepatobiliary malignancy
- 21. Thank you