Chapter 23: Sclerosing Cholangitis

INTRODUCTION

• Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease characterized by inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to the formation of multifocal bile duct strictures, biliary stasis, progressive cholestasis, and eventually the development of liver cirrhosis, portal hypertension, and hepatic decompensation (Figs. 23-1 to 23-5).¹

• The mean interval from diagnosis of PSC to death is 12 to 18 years, resulting mainly from infectious cholangitis and sepsis, complications of cirrhosis, and cholangiocellular carcinoma (CC).

• CC develops in 13% to 14% of patients, and in half of them is diagnosed simultaneously or within 1 year of PSC diagnosis (Figs. 23-6 and 23-7).

• PSC is strongly associated with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC), in 60% to 70% of patients.²

• PSC patients have an increased global risk of developing malignancies that range from 40% to 58%, including not only cholangiocarcinoma but also mainly colorectal, gallbladder, and hepatocellular carcinoma.³

• The etiology of PSC is unclear, and even though it is thought that the disease may have an autoimmune origin, it usually responds unfavorably to immunosuppressive therapy.^1,2

• Effective medical management has not been developed, and there is still no evidence supporting a specific treatment capable of halting disease progression. Even data on the impact of ursodeoxycholic acid on the course of the disease and survival remains unclear.²

• Management remains focused on treating the symptoms and complications associated with PSC progression, as well as close surveillance of advanced liver disease and cancer.²

• These patients frequently require endoscopic and/or surgical interventions for cholestatic manifestations resulting from dominant strictures (Fig. 23-8).⁴

• Although balloon dilation or stenting may help alleviate some of the symptoms associated with PSC, the only curative form of therapy for patients that progress to end-stage liver disease is orthotopic liver transplantation (OLT) (Fig. 23-9).^2,5

• Transplantation outcome is highly successful, and it has been reported to have the highest patient survival rate in comparison with other etiologies, with 5-year survival rates ranging from 83% to 88% (Fig. 23-10).⁵