Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by inflammation and fibrosis of the bile ducts that can progress to cirrhosis and liver cancer. It has a median survival time of 15 years and is the fifth most common reason for liver transplantation. PSC commonly affects males in their 40s and is associated with inflammatory bowel disease. Diagnosis involves clinical features, blood tests showing cholestasis, and imaging or biopsy of the bile ducts. Treatment includes medications like ursodeoxycholic acid and antibiotics, as well as endoscopic procedures and ultimately liver transplantation for advanced disease.

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)

4. Association of diseases

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