Background: Sepsis associated cholestasis in intensive care patients usually subsides with treatment of the underlying cause. However, in a subgroup of these patients a progressive destruction of intrahepatic bile ducts and sclerosing cholangitis with the typically appearance of segmental strictures and cholangiectasies develops. In this respect, a progressive form of sclerosing cholangitis after septic shock and long term intensive care treatment has been described. We herein report the clinical course and endoscopic treatment in patients with sclerosing cholangitis due to critical illness. Patients: Twelve patients (6 male, age: 48.8+12.7, (mean, years+SD)) with sclerosing cholangitis, diagnosed by endoscopic retrograde cholangiopancreaticography were identified. No patient had evidence of preexisting hepato-biliary disease or inflammatory bowel disease. Transfer to the ICU was necessary because of sepsis in 2, extensive surgery in 4, polytrauma in 2, cardiopulmonary resuscitation in 2 and respiratory failure in 2 patients, respectively. All patients needed mechanical ventilation, had a preexisting pulmonary disease and/or developed a severe adult respiratory distress syndrome. Results: Cholestasis or jaundice developed 60 [13-110] days (median[range]) after transfer to the ICU. The laboratory findings were: Bilirubin: 7.5[0.4-62.1], Alkaline Phosphatase: 10.6[2.6-22.5], GGT: 34.0[10.3-91.4], ALT: 2.0[0.8-18.7], AST: 3.6[0.8-34.0],(xULN, median[range]). ERCP demonstrated sclerosing cholangitis in all patients. Severe ductopenia was seen in two patients. In 9 patients endoscopic sphincterotomy was performed. Casts and sludge were extracted in 5 patients. Biliary stenting was performed in one patient with a dominant stenosis. Two patients were lost for follow up. During a median follow up of 11 [4–46] months 7 patients (58%) progressed to liver cirrhosis. Two patients died of liver failure and 2 patients underwent orthotopic liver transplantation. Stabilization or biochemical improvement of cholestasis was achieved in 3 patients. Discussion: Reduced hepatic oxygen delivery may lead to initial bile duct injury in ICU patients. As a second hit septicemia, ischemia, translocation of endotoxins from the gut may further lead to progressing sclerosing cholangitis. Long term outcome of endoscopic treatment is poor as a high proportion of these patients develop liver cirrhosis, for whom liver transplantation should be considered. Background: Sepsis associated cholestasis in intensive care patients usually subsides with treatment of the underlying cause. However, in a subgroup of these patients a progressive destruction of intrahepatic bile ducts and sclerosing cholangitis with the typically appearance of segmental strictures and cholangiectasies develops. In this respect, a progressive form of sclerosing cholangitis after septic shock and long term intensive care treatment has been described. We herein report the clinical course and endoscopic treatment in patients with sclerosing cholangitis due to critical illness. Patients: Twelve patients (6 male, age: 48.8+12.7, (mean, years+SD)) with sclerosing cholangitis, diagnosed by endoscopic retrograde cholangiopancreaticography were identified. No patient had evidence of preexisting hepato-biliary disease or inflammatory bowel disease. Transfer to the ICU was necessary because of sepsis in 2, extensive surgery in 4, polytrauma in 2, cardiopulmonary resuscitation in 2 and respiratory failure in 2 patients, respectively. All patients needed mechanical ventilation, had a preexisting pulmonary disease and/or developed a severe adult respiratory distress syndrome. Results: Cholestasis or jaundice developed 60 [13-110] days (median[range]) after transfer to the ICU. The laboratory findings were: Bilirubin: 7.5[0.4-62.1], Alkaline Phosphatase: 10.6[2.6-22.5], GGT: 34.0[10.3-91.4], ALT: 2.0[0.8-18.7], AST: 3.6[0.8-34.0],(xULN, median[range]). ERCP demonstrated sclerosing cholangitis in all patients. Severe ductopenia was seen in two patients. In 9 patients endoscopic sphincterotomy was performed. Casts and sludge were extracted in 5 patients. Biliary stenting was performed in one patient with a dominant stenosis. Two patients were lost for follow up. During a median follow up of 11 [4–46] months 7 patients (58%) progressed to liver cirrhosis. Two patients died of liver failure and 2 patients underwent orthotopic liver transplantation. Stabilization or biochemical improvement of cholestasis was achieved in 3 patients. Discussion: Reduced hepatic oxygen delivery may lead to initial bile duct injury in ICU patients. As a second hit septicemia, ischemia, translocation of endotoxins from the gut may further lead to progressing sclerosing cholangitis. Long term outcome of endoscopic treatment is poor as a high proportion of these patients develop liver cirrhosis, for whom liver transplantation should be considered.
Read full abstract