Secondary sclerosing cholangitis

Last revised by Francis Deng on 30 Jan 2020

Secondary sclerosing cholangitis refers to manifestations of sclerosing cholangitis that resemble primary sclerosing cholangitis but can be attributed to known insults to the biliary tree.


Secondary sclerosing cholangitis is a chronic cholestatic disease. The entity is characterized by inflammation and obliterative fibrosis of the bile ducts.


The main causes may be categorized into three mechanistic categories 1,2:

  • chronic biliary obstruction leading to recurrent suppurative cholangitis
  • ischemic cholangiopathy
    • liver allograft hepatic artery thrombosis
    • liver allograft rejection
    • intra-arterial chemotherapy or embolization
    • vasculitis
    • radiation therapy
    • critically ill patients 3
  • infectious cholangitis, especially AIDS cholangiopathy
    • cryptosporidiosis
    • cytomegalovirus

Although sometimes considered as part of primary or secondary sclerosing cholangitis, IgG4-related sclerosing cholangitis is a distinct entity responsive to glucocorticoids and is discussed separately.

Radiographic features

Bile duct imaging can be performed: magnetic resonance cholangiopancreatography (MRCP) 4, endoscopic retrograde cholangiopancreatography (ERCP), or percutaneous transhepatic cholangiography (PTC)

The appearance is generally similar to that of primary sclerosing cholangitis: the contours of the intrahepatic and extrahepatic biliary tree are irregular, with areas of stricturing or dilatation. Filling defects may be present, representing casts, sludge, and/or stones.

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