A 36-year-old woman presents with a 6-month history of progressive generalized itching, weight loss, fatigue, and yellow sclerae. She denies use of oral contraceptives or any other medication. Physical examination reveals mild jaundice and steatorrhea. Blood studies show a high cholesterol level of 350 mg/dL, elevated serum alkaline phosphatase (240 U/L), and normal levels of AST and ALT. An intravenous cholangiogram shows no evidence of obstruction. An antimitochondrial antibody test is positive; antinuclear antibodies are not present. For the patient, a liver biopsy would most likely show which of the following pathologic findings?
Primary biliary cirrhosis (nonsuppurative destructive cholangitis) is caused by chronic destruction of intrahepatic bile ducts in the portal tracts. Primary biliary cirrhosis evolves through ductal lesions, scarring, and eventually cirrhosis. Early PBC features chronic destructive cholangitis affecting intrahepatic small and medium-sized bile ducts. The bile ducts are surrounded primarily by lymphocytes (CD8+ T cells), but plasma cells and macrophages are also seen. In some portal tracts, lymphoid follicles are conspicuous. Discrete epithelioid granulomas often occur in the portal tracts and may impinge on the bile ducts. As a result of the destructive chronic inflammatory process, small bile ducts virtually disappear, and scarring of medium-sized bile ducts is common. Proliferation of bile ductules within portal tracts is common and may be florid. Collagenous septae extend from the portal tracts into the lobular parenchyma and encircle some lobules. Cholestasis, when present, may be severe and is located at the periphery of the portal tracts. The end-stage of PBC is cirrhosis, characterized by a dark green bile-stained liver that exhibits fine nodularity. The other choices do not feature destruction of intrahepatic bile ducts.Diagnosis: Primary biliary cirrhosis
Central hyaline sclerosis
Cholangiocarcinoma
Hemosiderosis
Intrahepatic bile duct damage
Liver & Biliary Tract