Secundary sclerosing cholangitis is an important clinical entity in critically ill patients (SCCIP) with severe polpyraumas and shock symptoms. Most current data come from transplant centers at the end stage of the disease. The aim of this study was to indentify all patients with SC-CIP, to characterize imaging modalities such as US,MS-CT,MRCP, EUS and ERC for the diagnosis and therapy (ERC). We included 57 patients (m=41, f=16, age median 53y) from a large Bavarian trauma center from 2/2005-11/2009 retrospective (n=28) and 12/2009 -11/2011 prospective (n=29). SC-CIP-diagnosis of all patients (n=57) was confirmed by ERC by typical bile duct changes and by extraction and histology of cast material. Underlying disorders were 26 (46%) intracerebral bleeding, 17 (30%) severe polytrauma with MOF, 10 (17%) with severe septic schock, 4 (7%) with severe burning . At admission all patients except one had a NACA score . 3 and a SOFA score of median 8 (63.5% of the patients . 7). Intensive care stay lasted 44 days (range 23-298 days). SOFA score further increased to 11 on day 4 of admission. Patients had normal liver enzymes and gGT (median 32 U/l ) and AP values at admission and on day 1-4 of ICU stay. There was a characteristic rise of gGT on day 6 (median 98 U/l) reaching maximum values on days 11-13 (median 669 U/l). AP showed a linear increase also starting on day 5, whereas liver enzymes and bilirubin in median increased gradually. Abdominal US initially showed no dilation of the external or internal bile ducts, but showed intraluminal reflexes. With time intrahepatic segmental dilatation occurred. EUS showed echodense material with typical double-linear contours, characterized as cast material by ERC. ERC was performed median on day 53 of the ICU stay. In 35 (60%) of the patients both sides of the biliary systems were affected, whereas in 23 patients only one side was affected. In all patients papillotomy was performed and cast material was removed when present, strictures of the main duct and both hepatici were balloon dilated, all patients received antibiotics. 51/57 patients (89%) were followed by routine follow up, readmission, telephone contact or questionnaire with a follow up of 34.7 month. 27 patients had a favorable (n=8) or stable course (n=19). 3 patients received LTX, 5 patients have a negative course of the liver disease being listed for LTX, 16 Patients died during follow up, 4/16 died as a consequence of the SC-CIP. In conclusion: This study describes in a prospective manner in a single center study the clinical signs, course and parameters of SC-CIP patients. Mandatory interventional ERC removed biliary casts and stricutes and determined a long term outcome (2,6 years) with a letality of 14.5%, LTX rate of 5.1% and listing for LTX of 8.6%. This study adds new important clinical information to the initial course of this disease.