Clinical features:
(1) history of admission to the intensive care unit (ICU)
(2) aggressive treatment including mechanical ventilation and pressor therapy
(3) negative past history for biliary obstruction, primary sclerosing cholangitis or other biliary disorder
(4) often rapid disease progression with biliary cirrhosis and the need for liver transplantation
Pathologic features may include some combination of:
(1) development of protein-rich casts in biliary ducts (which can be seen on ERCP)
(2) secondary infection with bacteria, often antibiotic-resistant
(3) ischemic cholangiopathy
The diagnosis requires exclusion of:
(1) primary sclerosing cholangitis (PSC)
(2) other causes of secondary sclerosing cholangitis.
(3) development of strictures in the main bile ducts
