Pathology - Primary Sclerosing Cholangitis
Believed to be autoimmune in nature; 60%-80% of cases are linked to inflammatory bowel disease, typically ulcerative colitis. Prevalent among males aged 20 to 40. Pathology Gross: Liver shows a progressive green tint. Microscopic findings include fibrosis surrounding bile ducts known as "onion skin fibrosis" along with a lymphocytic infiltration. Fibrosis leads to narrowing or blockage of the bile ducts, causing cholestasis and eventually biliary cirrhosis. Clinical Symptoms Symptoms of obstructive jaundice include itching, fatigue, unintentional weight loss, fatty stools due to poor absorption, and enlargement of the liver and spleen. Complications may arise such as the formation of SBC leading to portal hypertension and a higher occurrence of cholangiocarcinoma. Laboratory results: Elevated ALP and bilirubin levels; p-ANCA antibodies present in 80% of patients. Treatment includes Ursodeoxycholic acid, vitamin supplements, symptomatic treatment of biliary obstruction by endoscopic dilatation, and liver transplant for severe illness. Notes
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