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.

Persistent Cholestasis After Critical Illness

“…After a few months, there is a progressive fibrosis of the intrahepatic bile ducts with bile duct proliferation, portal inflammatory infiltrates, periductal fibrosis, and canalicular cholestasis [3,4,7].…”

“…The biliary epithelium receives blood supply exclusively from peribiliary plexus of the hepatic artery, in contrast to the hepatic parenchyma which has a dual blood supply from the hepatic artery and the portal vein. Therefore, the biliary epithelium is more susceptible to ischemia than hepatocytes [2,3].…”

“…Clinical signs are non-specific and cholestatic derangement of liver function persists despite recovery from the primary disease that differentiates patients with SC-CIP from patients with endotoxin-associated jaundice, which resolves after clinical recovery [3,5]. Imaging or endoscopic retrograde cholangiography (ERCP) diagnoses biliary casts and detects the biliary stricture formation.…”

Leberwerterhöhung bei einem Intensivpatienten

“…Es kommt zu einer relativ schnellen Krankheitsprogression mit Entwicklung einer biliären Leberzirrhose und sehr schlechter Prognose [3]. Bei nachlassender Syntheseleistung der Leber oder Zeichen der portalen Hypertension sollte die Evaluation zur Lebertransplantation erfolgen.…”

How to assess liver function?

“…Some of these patients will, however, after surviving the acute phase of critical illness, develop secondary sclerosing cholangitis with rather rapid progression to biliary fibrosis, cirrhosis and liver failure. This rare, but increasingly recognized, problem of sclerosing cholangitis is a dismal disease, resulting in death without liver transplantation in the majority of cases [12 ].…”