Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an
important differential diagnosis in patients presenting with cholestasis and
PSC-like cholangiographic changes in endoscopic retrograde cholangiography
(ERC). As a relatively newly described entity, SSC-CIP is still
underdiagnosed, and the diagnosis is often delayed. The present study aims to
improve the early detection of SSC-CIP and the identification of its
complications. A total of 2633 records of patients who underwent or were
listed for orthotopic liver transplantation at the University Hospital
Charité, Berlin, were analyzed retrospectively. The clinical presentation and
outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were
reviewed. Cholestasis was the first sign of SSC-CIP. GGT was the predominant
enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the
patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our
patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different
cholangiographic features detected correspond roughly to the following stages:
(I) evidence of biliary casts, (II) progressive destruction of intrahepatic
bile ducts, and (III) picture of pruned tree. Biliary cast formation is a
hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients,
the clinical course was complicated by cholangiosepsis, cholangitic liver
abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was
associated with worse prognosis; transplant-free survival was ∼40 months
(mean). Because of its high rate of serious complications and unfavorable
prognosis, it is imperative to diagnose SSC-CIP early and to differentiate
SSC-CIP from other types of sclerosing cholangitis. Specific characteristics
enable identification of SSC-CIP. Early cooperation with a transplant center
and special attention to biliary complications are required after diagnosis of
SSC-CIP