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.
Pathology
Secondary sclerosing cholangitis is a chronic cholestatic disease. The entity is characterized by inflammation and obliterative fibrosis of the bile ducts.
Etiology
The main causes may be categorized into three mechanistic categories 1,2:
- chronic biliary obstruction leading to recurrent suppurative cholangitis
- choledocholithiasis
- surgical biliary trauma
- chronic pancreatitis
- biliary neoplasm, such as cholangiocarcinoma
- liver allograft biliary anastomotic stricture
- 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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