title: Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases.
abstract: 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.
authors.affiliation: From the Department of General, Visceral and Transplantation Surgery, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin, Germany (SL, DE, WF, PN, DS); Endoscopy Unit, Department of Gastroenterology and Hepatology, Endocrinology, Diabetes and Metabolic Diseases, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin, Germany (WV-S, AA, ES); and Department of Gastroenterology, Hepatology and Infectious Diseases, Heinrich-Heine-University Düsseldorf, Moorenstrasse 5, Düsseldorf, Germany (SL).
authors.forename: Silke
authors.initials: S
authors.lastname: Leonhardt
authors.forename: Wilfried
authors.initials: W
authors.lastname: Veltzke-Schlieker
authors.forename: Andreas
authors.initials: A
authors.lastname: Adler
authors.forename: Eckart
authors.initials: E
authors.lastname: Schott
authors.forename: Dennis
authors.initials: D
authors.lastname: Eurich
authors.forename: Wladimir
authors.initials: W
authors.lastname: Faber
authors.forename: Peter
authors.initials: P
authors.lastname: Neuhaus
authors.forename: Daniel
authors.initials: D
authors.lastname: Seehofer
mesh_terms: D000328:Adult; D001653:Bile Ducts, Intrahepatic / Q000503:physiopathology; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D002779:Cholestasis / Q000503:physiopathology; D016638:Critical Illness; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006937:Hypercholesterolemia / Q000503:physiopathology; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D015431:Weight Loss