Sekundär sklerosierende Cholangitis bei kritisch Kranken

Zusammenfassung

Die 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.

Abstract

Sclerosing Cholangitis of the Critically Ill (SC-CIP) is a relatively new entity within the spectrum of secondary cholangitis that develops in the wake of intensive care therapy with mechanical ventilation and catecholamine treatment. It is caused by ischemic or immunologic injury to small bile ducts that becomes self-aggravating and persists beyond the end of the intensive care stay. Early clinical and laboratory findings show acute cholangitis with elevated CRP, gamma GT, AP, and bilirubin. ERCP shows damaged intrahepatic bile ducts with irregular calibers and biliary casts. The following phase is chronic and oligosymptomatic. Still, all laboratory parameters will stay mildly elevated and ERCP and MRCP will show progressive loss of small bile ducts. Long-term prognosis is poor. Even with UDCA therapy, most patients will develop liver cirrhosis within months or years.

Schlüsselwörter

Sekundär sklerosierende Cholangitis - Intensivtherapie - intrahepatische Gallenwege - Leberzirrhose

Key words

secondary sclerosing Cholangitis - intensive care - intrahepatic bile ducts - liver cirrhosis

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