This case illustrates the typical pathological features and disease course in primary biliary cirrhosis. This case also illustrate the role of the pathologist in diagnosis and treatment. A 46 year old woman complains of generalized skin itching and fatigue. Her medical history includes hypothyroidism, for which she takes thyroid hormone replacement.
Physical examination reveals an enlarged liver with a firm edge and palpable spleen.
Alkaline phospahatase is increased out of proportion to the other liver enzymes in cholestatic disorders. Abdominal ultrasound shows hepatomegaly and splenomegaly, with no dilatation of the bile ducts or gallstones. A heavy infiltrate of lymphocytes and plasma cells surrounds a bile duct with epitelial damage. Noncaseating granuloma at edge of portal tract. Eighteen months later she develops jaundice, and after four more years, uncontrollable itching, ascites, and mild encephalopathy with a poor quality of life.