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Primary Sclerosing Cholangitis (A chronic cholestatic disorder, Involves…
Primary Sclerosing Cholangitis
A chronic cholestatic disorder
Involves extrahepatic and large intrahepatic bile ducts
Progressive fibrosis and destruction of extrahepatic and large intrahepatic bile ducts
Common in association with IBD (esp. UC) UC present in 70% of patients with PSC, while PSC present in 4% of patients with UC
P-ANCA is present in 80% of cases. indicate that it is an autoimmune Ds
Age: 3rd to 5th decades
M:F is 2:1
Insidious onset
Progressive fatigue, pruritus(itchy skin) , and jaundice
Chronic liver disease late in the course (when fibrosis and obstruction occurs)
Cholangiocarcinomamay develop in 10-15% of individuals (median time of 5 years from diagnosis)
Retrograde cholangiography shows a characteristic “beaded appearance
Fibrosing cholangitis of bile ducts
periductal onion-skin fibrosis & lymphocytic infiltrate
Sclerosing means: A thickening or hardening of a body part due to excessive fibrosis
Cholangitis: it involve minor inflammation, so there is a
few inflammatory cells
Progressive fibrosis and destruction
Common in association with IBD UC present in 70% of patients with PSC
Cholangiocarcinoma: which is a carcinoma arising from the epithelial cells lining
the biliary system, and it is a very poor prognostic carcinoma
PSC has a protracted (elongated) course over years
P 7 + 8
Characterized by