This condition is characterized by multiple intrahepatic and extrahepatic biliary strictures associated with areas of dilatation. Where gallstones or another hepatobiliary disease co-exist it is presumed to be the result of cholangitis, and therefore deemed to be "secondary". Sacculation of the large ducts, a characteristic feature, is well shown in Fig. 4.118. The histopathological features of primary sclerosing cholangitis appear in Fig. 4.122b.
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