secondary sclerosing cholangitis
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 :
- 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
- 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) , 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.