Secondary sclerosing cholangitis in a critically ill patient

Weiss, Krista E.; Willmann, Jürgen K.; Jeffrey, R. Brooke; Desser, Terry S.

doi:10.21037/qims.2016.04.04

Cited by 3 publications

(4 citation statements)

References 19 publications

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“…While ultrasound imaging can be undertaken at the bedside, it is operator dependent and cannot always be relied on in the diagnosis of intrahepatic biliary diseases; while some authors report visible changes within the hepatobiliary complex in accordance with sclerosing cholangitis, 29 others have reported unremarkable ultrasound imaging 30 in keeping with the case discussed. MRCP or ERCP are more likely to aid the diagnosis of SSC-CIP, but these investigations are often delayed due to patients not being sufficiently stable to be moved out of the critical care environment.…”

Section: Discussionmentioning

confidence: 83%

Secondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection

2020

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A previously well 59-year-old man required a prolonged intensive care unit stay due to severe COVID-19 symptoms. During the admission, he developed a cytokine storm, also known as secondary haemophagocytic lymphohistocytosis, and multiorgan failure. Despite recovering from his other organ failures, his liver function continued to deteriorate. Magnetic resonance cholangiopancreatography and subsequent endoscopic retrograde cholangiopancreatography revealed extensive intrahepatic duct dilatation with ‘beading’ but common bile duct sparing. Given the patient had no primary liver disease prior to admission, we considered secondary causes of cholestatic liver injury; this led us to an unusual diagnosis of secondary sclerosing cholangitis in critically ill patients. This case demonstrates a rare disease that has developed specifically in the context of SARS-CoV-2 infection. A review of current literature and the underlying pathophysiology for this rare disease are discussed, particularly in relation to COVID-19.

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Section: Discussionmentioning

confidence: 83%

Secondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection

2020

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“…These are followed by infections that can lead to multiple hepatic abscesses. In later stages, findings are multiple irregular strictures, dilations, beading, wall thickening, and destruction of the intrahepatic bile ducts with sparing of the common bile duct forming a “pruned tree” appearance 18,24,41. All patients have intrahepatic bile duct involvement, whereas only around a fifth of patients also have minor extrahepatic disease 18.…”

Section: Introductionmentioning

confidence: 99%

“…In later stages, findings are multiple irregular strictures, dilations, beading, wall thickening, and destruction of the intrahepatic bile ducts with sparing of the common bile duct forming a “pruned tree” appearance. 18 , 24 , 41 All patients have intrahepatic bile duct involvement, whereas only around a fifth of patients also have minor extrahepatic disease. 18 Leonhardt et al 18 found out that a total of 88% of SSC-CIP patient developed imaging features of liver cirrhosis within 6 months of diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Secondary sclerosing cholangitis in critically ill patients: current perspectives

Gudnason¹,

Bjõrnsson²

2017

CEG

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Secondary sclerosing cholangitis (SSC) is a term used for a group of chronic cholestatic disease affecting the intra- and/or extrahepatic biliary tree with inflammation and progressive stricture formation, which can lead to biliary cirrhosis. A newly recognized form of SSC is secondary sclerosing cholangitis in critically ill patients (SSC-CIP). Pathogenesis is believed to involve ischemic injury of intrahepatic bile ducts associated with prolonged hypotension, vasopressors administration, and/or mechanical ventilation in patients treated in the intensive care unit (ICU). Patients diagnosed with SSC-CIP have no prior history of liver disease and no known pathologic process or injury responsible for bile duct obstruction prior to ICU treatment. Reasons leading to ICU treatment are many including multitrauma, burn injury, cardiac surgery, severe pneumonia, other infections, or bleeding after abdominal surgery. Patients have in common prolonged ICU admission. SSC-CIP is associated with rapid progression to liver cirrhosis and poor survival with limited treatment options except a liver transplantation. Transplant-free survival is around 17–40 months, which is lower than in other SSC patients. During the initial stages of the disease, the clinical symptoms and biochemical profile are not specific and easily missed. Biliary casts formation may be considered pathognomonic for SSC-CIP since most patients have them in early stages of the disease. Increased awareness and early detection of the disease and its complications is considered to be crucial to improve the poor prognosis.

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“…Typische ERC-Befunde in einem frühen Krankheitsstadium sind vielfache bandförmige Füllungsdefekte der kleinen und auch großen Gallenwege. Diese entsprechen biliären Ausgusssteinen ("casts") [6,27] periphere Dilatationen, Wandverbreiterungen und eine generelle Zerstörung der intrahepatischen Gallenwege ("vanishing bile ducts") nachweisen [7,28]. Während die intrahepatischen kleinen Gallenwege bei allen Patienten betroffen sind, finden sich extrahepatische Veränderungen eher selten.…”

Section: Klinisches Bild Labor Verlaufunclassified

Sekundär sklerosierende Cholangitis bei kritisch Kranken

2019

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ZusammenfassungDie sklerosierende Cholangitis der kritisch Kranken (secondary cholangitis of the critically ill patient: SC-CIP) ist eine relativ neue Entität innerhalb der sekundären Cholangitiden. Sie entwickelt sich in der Folge einer intensivmedizinischen Behandlung mit Beatmung und Katecholamintherapie. Pathophysiologische Grundlage ist eine ischämische oder immunologische Schädigung der intrahepatischen Gallenwege, die sich verselbstständigt und über das Ende der Intensivtherapie hinaus fortbesteht. Klinisch und laborchemisch findet sich zunächst eine akute Cholangitis mit einem Anstieg von CRP, gamma-GT, AP und Bilirubin. Die ERCP zeigt Schäden an den kleinen Gallenwegen mit Kalibersprüngen und biliären Ausgusskonkrementen. Die darauf folgende chronische Phase verläuft oligosymptomatisch. Alle Laborparameter bleiben aber leicht erhöht; in ERCP und MRCP zeigt sich ein fortschreitender Untergang der intrahepatischen Gallenwege. Die Langzeitprognose ist schlecht. Auch unter einer Therapie mit UDCA entwickelt die Mehrzahl der Patienten innerhalb von Monaten bis wenigen Jahren eine Leberzirrhose.

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Secondary sclerosing cholangitis in a critically ill patient

Cited by 3 publications

References 19 publications

Secondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection

Secondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection

Secondary sclerosing cholangitis in critically ill patients: current perspectives

Sekundär sklerosierende Cholangitis bei kritisch Kranken

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