2 BenignNontraumaticAbnormalities 38 Primary Biliary Cirrhosis Related topics: 36, 37 (cirrhosis) KEY FACTS: DISEASE Progressive destructive cholangitis of interlobar and septal bile ducts with inflammatory cellular infiltrate, portal fibrosis, nodular regeneration, and shrinkage of hepatic parenchyma Presenting age: 35 55 years Ratioofwomentomen:9:1 Etiology:immuneresponsedisorder Associated diseases: rheumatoid arthri- tis, Sjögrens syndrome, Hashimotos thyroiditis, dermatomyositis, systemic lupus erythematosus Clinical signs: fatigue, pruritus, kerato- conjunctivitis sicca, hyperpigmentation Tests for antimitochondrial antibodies usually positive ( 90 ) Differentialdiagnosiswithprimaryscle- rosing cholangitis (see 41): sex distri- bution, associated diseases, cholangio- graphic findings (see below) Complications:hepatocellularcarcinoma, liver insufficiency KEY FACTS: MRI Earlystage:noabnormalities Advanceddisease(Fig.
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