Info
🌱 來自: Huppert’s Notes
Cholestatic Liver Diseases🚧 施工中
Cholestatic Liver Diseases
Primary biliary cholangitis (PBC)
• Etiology: Immune-mediated destruction of intrahepatic bile ducts, most common in females 40–50 yr
• Clinical features: Fatigue, pruritus, jaundice
• Diagnosis: At least two of following: AlkP >1.5× ULN, +anti-mitochondrial antibody, liver biopsy with destruction of interlobular bile ducts and destructive cholangitis
• Treatment: First line: ursodiol slows progression. Second line: obeticholic acid in compensated cirrhosis. For advanced disease, liver transplantation.
Primary sclerosing cholangitis (PSC)
• Etiology: Fibroinflammatory disorder of bile ducts, most common in men 20–30 yr. 80% patients have IBD (UC)
• Clinical features: Patients are usually asymptomatic or may develop pruritus, abdominal pain, cholangitis
• Diagnosis: MRCP or ERCP: “Beads on a string” of intrahepatic and extrahepatic ducts. Liver biopsy: Fibrous obliteration of the small bile ducts with an “onion skin” appearance on pathology
• Treatment: No effective treatment. Progressive disease that requires liver transplantation. High association with cholangiocarcinoma: Screen with q6–12mo LFTs, Ca19-9, MRCP