Secondary sclerosing cholangitis

ABSTRACT Secondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and

progressive destruction of the biliary tree that leads to biliary cirrhosis. SSC is thought to develop as a consequence of known injuries or secondary to pathological processes of the

biliary tree. The most frequently described causes of SSC are longstanding biliary obstruction, surgical trauma to the bile duct and ischemic injury to the biliary tree in liver allografts.

SSC may also follow intra-arterial chemotherapy. Sclerosing cholangitis in critically ill patients is a largely unrecognized new form of SSC, and is associated with rapid progression to

liver cirrhosis. The mechanisms leading to cholangiopathy in critically ill patients are widely unknown; however, the available clinical data indicate that ischemic injury to the

intrahepatic biliary tree may be one of the earliest events in the development of this severe form of sclerosing cholangitis. Therapeutic options for most forms of SSC are limited, and

patients with SSC who do not undergo transplantation have significantly reduced survival compared with patients with primary sclerosing cholangitis. Sclerosing cholangitis in critically ill

patients, in particular, is associated with rapid disease progression and poor outcome. KEY POINTS * Secondary sclerosing cholangitis (SSC) may be caused by various insults to the biliary

tree; main causes are chronic biliary obstruction, infectious or toxic cholangitis, immunological causes and ischemic cholangiopathies * SSC is a progressive disease characterized by

fibrosis and destruction of the biliary tree, which leads to biliary cirrhosis; in most cases, the benefits of therapeutic interventions are limited * Sclerosing cholangitis in critically

ill patients (SC–CIP) is a new entity of sclerosing cholangiopathy and is associated with a particularly rapid progression of the disease * The hallmark of SC–CIP is the early formation of

biliary casts; the etiology of SC–CIP may involve early ischemic bile duct injury; however other factors are probably also involved * The clinical signs during the initial phase of SC–CIP

are not specific; a diagnosis of SC-CIP is often overlooked and, therefore, the frequency of SC–CIP is probably underestimated * Aside from transplantation, there are no effective treatment

options for SC–CIP; the median survival of patients with SC–CIP who do not undergo liver transplantation is only about 13 months Access through your institution Buy or subscribe This is a

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SCLEROSING CHOLANGITIS Article 13 March 2025 PRIMARY SCLEROSING CHOLANGITIS (PSC) AND INFLAMMATORY BOWEL DISEASE (IBD): A CONDITION EXEMPLIFYING THE CROSSTALK OF THE GUT–LIVER AXIS Article

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P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited

continuing medical education activity associated with this article. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Internal Medicine I, University of Regensburg, Germany Petra

Ruemmele, Ferdinand Hofstaedter & Cornelia M. Gelbmann Authors * Petra Ruemmele View author publications You can also search for this author inPubMed Google Scholar * Ferdinand

Hofstaedter View author publications You can also search for this author inPubMed Google Scholar * Cornelia M. Gelbmann View author publications You can also search for this author inPubMed 

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Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Ruemmele, P., Hofstaedter, F. & Gelbmann, C. Secondary sclerosing cholangitis. _Nat Rev Gastroenterol Hepatol_ 6, 287–295

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