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Pathology - Primary sclerosing cholangitis
Definition: A chronic hepatic condition marked by inflammation and fibrosis inside the biliary tree. The entire biliary tree is typically involved; but, in some instances, just the small interlobular bile ducts are impacted, known as small duct primary sclerosing cholangitis (PSC).
Epidemiology • Rare occurrence. • Primarily observed in young males with ulcerative colitis (about 70% of patients with primary sclerosing cholangitis also have ulcerative colitis).
Aetiology • Unknown, though a genetic association with specific HLA types exists.
Pathogenesis: Chronic biliary inflammation leads to fibrotic scarring that constricts the afflicted bile ducts. Obstruction in the biliary system results in gradual fibrosis of the liver, culminating in cirrhosis. Biliary stasis additionally facilitates infection and the production of stones.
Presentation • Typically asymptomatic in first stages, but frequently detected through high alkaline phosphatase values in patients with known ulcerative colitis (UC).
Radiology • The presence of strictures and dilations in the biliary tree observed on imaging is strongly indicative of primary sclerosing cholangitis (PSC).
Macroscopy • Initial PSC typically results in no observable macroscopic alterations. Progressive illness results in a cirrhotic liver with bile stains. Fibrotic biliary strictures may be evident in the principal bile ducts.
Histopathology • Explanted liver specimens exhibit fibrosis and inflammation in the major bile ducts, accompanied by thickened bile and calculi. A biliary pattern of cirrhosis is characterized by huge, uneven, jigsaw-like nodules of hepatocytes. Liver biopsy results exhibit varied features based on the biopsy location. If the biopsy is obtained from a region not impacted by the underlying disease, however distal to a significant duct stricture, the liver exhibits characteristics of duct obstruction (i.e., portal edema accompanied by bile ductule growth). If the biopsy is obtained from a region impacted by PSC, medium-sized bile ducts have periductal edema and concentric fibrosis, while small bile ducts are frequently entirely absent.
Prognosis: Progressive hepatic illness ultimately resulting in cirrhosis. Two patients are at elevated risk for bile duct cancer, which occurs in around 20% of cases and is associated with a dismal prognosis.
Definition: A chronic hepatic condition marked by inflammation and fibrosis inside the biliary tree. The entire biliary tree is typically involved; but, in some instances, just the small interlobular bile ducts are impacted, known as small duct primary sclerosing cholangitis (PSC).
Epidemiology • Rare occurrence. • Primarily observed in young males with ulcerative colitis (about 70% of patients with primary sclerosing cholangitis also have ulcerative colitis).
Aetiology • Unknown, though a genetic association with specific HLA types exists.
Pathogenesis: Chronic biliary inflammation leads to fibrotic scarring that constricts the afflicted bile ducts. Obstruction in the biliary system results in gradual fibrosis of the liver, culminating in cirrhosis. Biliary stasis additionally facilitates infection and the production of stones.
Presentation • Typically asymptomatic in first stages, but frequently detected through high alkaline phosphatase values in patients with known ulcerative colitis (UC).
Radiology • The presence of strictures and dilations in the biliary tree observed on imaging is strongly indicative of primary sclerosing cholangitis (PSC).
Macroscopy • Initial PSC typically results in no observable macroscopic alterations. Progressive illness results in a cirrhotic liver with bile stains. Fibrotic biliary strictures may be evident in the principal bile ducts.
Histopathology • Explanted liver specimens exhibit fibrosis and inflammation in the major bile ducts, accompanied by thickened bile and calculi. A biliary pattern of cirrhosis is characterized by huge, uneven, jigsaw-like nodules of hepatocytes. Liver biopsy results exhibit varied features based on the biopsy location. If the biopsy is obtained from a region not impacted by the underlying disease, however distal to a significant duct stricture, the liver exhibits characteristics of duct obstruction (i.e., portal edema accompanied by bile ductule growth). If the biopsy is obtained from a region impacted by PSC, medium-sized bile ducts have periductal edema and concentric fibrosis, while small bile ducts are frequently entirely absent.
Prognosis: Progressive hepatic illness ultimately resulting in cirrhosis. Two patients are at elevated risk for bile duct cancer, which occurs in around 20% of cases and is associated with a dismal prognosis.
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