Sclerosing Cholangitis

Sclerosing cholangitis is a disease that affects the intra- and extrahepatic biliary system. Although it may have a variety of pathogenic origins, the histopathological changes are generally comparable. The biliary tracts, in particular, are affected by an intense inflammatory fibrosis [ 10].Additionally, enlarged lymphatic nodules localized in the liver hilum may be found. Although the hepatic parenchyma usually presents as normal, a fulminate form of sclerosing cholangitis may occur, lead ing to liver failure and portal hypertension [80].

Typical findings with MRI are diffusely distributed regions of biliary dilatation and areas of periportal inflammation. These features are best distinguished on heavily T2-weighted images when the biliary dilatation presents as areas showing a fluid-equivalent signal localized along branches of the portal veins. On the other hand, periportal inflammation shows decreased SI on T1-weighted images, while a signal intermediate between that of liver tissue and bile is seen on T2-weighted images. These imaging findings are typically localized in the liver hilum and accompany the intra-hepatic portal tracts surrounding the portal vein branches, but not the hepatic veins, which are unaffected by the disease. For the differential diagnosis of sclerosing cholangitis, periportal inflammation, biliary obstruction, hepatitis and periportal neoplasm all have to be taken into consideration.

Patients suffering from sclerosing cholangitis frequently develop an occult thrombosis of the main or segmental portal vein as a result of the periportal inflammatory process. This may subsequently lead to a segmental atrophy [9,28] (Fig. 4). Another important finding in sclerosing cholangi-tis is the increased risk of developing cholangio-carcinoma (CCC). For this reason imaging studies should always focus on the detection of focal lesions as well (Fig. 5).

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