Gelbmann Cornelia M, Rümmele Petra, Wimmer Monika, Hofstädter Ferdinand, Göhlmann Björn, Endlicher Esther, Kullmann Frank, Langgartner Julia, Schölmerich Jürgen
Department of Internal Medicine I and Institute of Pathology, University of Regensburg, Regensburg, Germany.
Am J Gastroenterol. 2007 Jun;102(6):1221-9. doi: 10.1111/j.1572-0241.2007.01118.x.
Sclerosing cholangitis in critically ill patients (SC-CIP) is a newly described entity of severe biliary disease with progression to liver cirrhosis. The mechanisms leading to this form of cholangiopathy with stricture formation and complete obliteration of bile ducts are unknown.
In the last 2 yr, sclerosing cholangitis was diagnosed in 26 patients during or after their stay on the intensive care unit by ERCP and/or liver histology. Complete patient records were available for 17 patients. Histological evaluations of liver biopsies and of four explanted livers, parameters of cardiovascular and respiratory conditions, treatment modalities, and accompanying infections were analyzed to find further hints for the pathomechanisms leading to SC-CIP.
With the beginning of cholestasis, the earliest endoscopic findings were intrahepatic biliary casts with impairment of the biliary flow and subsequent biliary infection, in most cases with Enterococcus faecium. Liver biopsy confirmed cholangitis and histology of explanted livers revealed ulcerated biliary epithelium with hemorrhagic exudates in the bile ducts. In the further course, progressive sclerosis with formation of multiple strictures of the bile ducts was observed. All patients suffered severe respiratory insufficiency with the need for mechanical ventilation (40.7+/-32.9 days). The PaO2/FiO2 ratio until beginning of cholestasis was 150.5+/-43.1. Half of the patients (9/17) were treated with high-frequency oscillatory ventilation and 12/17 patients by intermittent prone positioning. All patients required catecholamines for hemodynamic stabilization.
SC-CIP is a severe and in most cases rapidly progressive complication of intensive care patients. Ischemic injury of the biliary tree with the formation of biliary casts and subsequent ongoing biliary infection due to multiresistant bacteria seem to be major pathogenic mechanisms in the development of this new entity of sclerosing cholangitis.
危重症患者硬化性胆管炎（SC-CIP）是一种新描述的严重胆道疾病，可进展为肝硬化。导致这种伴有胆管狭窄形成和胆管完全闭塞的胆管病的机制尚不清楚。
在过去2年中，26例患者在重症监护病房住院期间或之后通过内镜逆行胰胆管造影（ERCP）和/或肝脏组织学检查被诊断为硬化性胆管炎。17例患者有完整的病历记录。分析肝脏活检组织学评估以及4例移植肝脏、心血管和呼吸状况参数、治疗方式及伴随感染情况，以寻找导致SC-CIP发病机制的更多线索。
胆汁淤积开始时，最早的内镜检查发现是肝内胆管铸型，伴有胆汁流动受损及随后的胆道感染，大多数情况下感染菌为粪肠球菌。肝脏活检证实为胆管炎，移植肝脏组织学显示胆管上皮溃疡伴胆管内出血性渗出物。在疾病进展过程中，观察到胆管进行性硬化并形成多个狭窄。所有患者均患有严重呼吸功能不全，需要机械通气（40.7±32.9天）。胆汁淤积开始前的动脉血氧分压/吸入氧分数值（PaO2/FiO2）为150.5±43.1。一半患者（9/17）接受高频振荡通气治疗，12/17患者采用间歇性俯卧位通气。所有患者均需要使用儿茶酚胺来维持血流动力学稳定。
SC-CIP是重症监护患者严重且多数情况下进展迅速的并发症。胆管树缺血性损伤伴胆管铸型形成以及随后因多重耐药菌导致的持续胆道感染似乎是这种新型硬化性胆管炎发病的主要致病机制。
