Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis is characterized by progressive fibro-inflammatory destruction of bile ducts, leading to stricturing and cholestasis. The pathophysiology remains incompletely understood but likely involves aberrant immune response to bile ducts, possibly triggered by microbial antigens or damage-associated molecular patterns. Intestinal dysbiosis and increased bacterial translocation appear to play roles. The strong association with inflammatory bowel disease suggests shared immune mechanisms. Genetic factors (HLA associations) and environmental factors (infections, toxins) likely contribute. PSC progresses from asymptomatic disease with abnormal liver enzymes to symptomatic disease with jaundice, pruritus, and cholangitis episodes. Bile duct stricturing leads to recurrent cholangitis (fever, jaundice, abdominal pain) requiring antibiotics and interventions. Progressive biliary obstruction leads to secondary biliary cirrhosis with portal hypertension, ascites, hepatic encephalopathy, and liver failure. PSC is a risk factor for cholangiocarcinoma (bile duct cancer), with 10-15% lifetime incidence. Disease progression varies widely, with median time to liver transplantation or death approximately 12-17 years from diagnosis. Diagnosis requires characteristic cholangiography showing bile duct stricturing and dilation. Currently, no medical therapy significantly delays progression, and treatment is primarily supportive and preventive (ursodeoxycholic acid, management of IBD, monitoring for cholangiocarcinoma).