PDF herunterladen
Z Gastroenterol 2019; 57(08): 977-982
DOI: 10.1055/a-0958-2843
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Sekundär sklerosierende Cholangitis bei kritisch Kranken

Secondary cholangitis of the critically ill
Florian Hentschel
  Zentrum für Innere Medizin II, Städtisches Klinikum Brandenburg GmbH, Brandenburg an der Havel, Germany
,
Till Bornscheuer
  Zentrum für Innere Medizin II, Städtisches Klinikum Brandenburg GmbH, Brandenburg an der Havel, Germany
,
Stefan Lüth
  Zentrum für Innere Medizin II, Städtisches Klinikum Brandenburg GmbH, Brandenburg an der Havel, Germany
› Institutsangaben
Weitere Informationen

Publikationsverlauf

27. Februar 2019

07. Juni 2019

Publikationsdatum:
09. August 2019 (online)

Auch verfügbar auf
    Lizenzen und Reprints

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
 

  • Literatur

  • 1 Schottmüller H. Cholangitis lenta. Münch med Wschr 1921; II: 1667
    PubMedGoogle Scholar
  • 2 Halawani A, Abdallah A. Cholangitis lenta; two cases. J Egypt Med Assoc 1954; 37 (10) 1205-1211
    PubMedGoogle Scholar
  • 3 Lefkowitch JH. Bile ductular cholestasis: an ominous histopathologic sign related to sepsis and “cholangitis lenta”. Hum Pathol 1982; 13: 19-24
    CrossrefPubMedGoogle Scholar
  • 4 Schmitt M, Kölbel CB, Müller MK. et al. Sklerosierende Cholangitis nach Verbrennung. Z Gastroenterol 1997; 35 (10) 929-934
    PubMedGoogle Scholar
  • 5 Gudnason HO, Björnsson ES. Secondary sclerosing cholangitis in critically ill patients: current perspectives. Clin Exp Gastroenterol 2017; 10: 105-111 . doi:10.2147/CEG.S115518
    CrossrefPubMedGoogle Scholar
  • 6 Leonhardt S, Veltzke-Schlieker W, Adler A. et al. Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases. Medicine (Baltimore) 2015; 94 (49) e2188
    PubMedGoogle Scholar
  • 7 Ben-Ari Z, Levingston D, Weitzman E. et al. Secondary sclerosing cholangitis following major burn. Ann Hepatol 2015; 14: 695-701
    CrossrefPubMedGoogle Scholar
  • 8 Engler S, Elsing C, Flechtenmacher C. et al. Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders. Gut 2003; 52: 688-693
    CrossrefPubMedGoogle Scholar
  • 9 Gelbmann CM, Rümmele P, Wimmer M. et al. Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients. Am J Gastroenterol 2007; 102: 1221-1229
    CrossrefPubMedGoogle Scholar
  • 10 Jaeger C, Mayer G, Henrich R. et al. Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up. Endoscopy 2006; 38: 730-734
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 11 Leonhardt S, Veltzke-Schlieker W, Adler A. et al. Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients. Crit Care 2015; 19: 131 . doi:10.1186/s13054-015-0861-5
    CrossrefPubMedGoogle Scholar
  • 12 Weig T, Schubert MI, Gruener N. et al. Abdominal obesity and prolonged prone positioning increase risk of developing sclerosing cholangitis in critically ill patients with influenza A-associated ARDS. Eur J Med Res 2012; 17: 30 . doi:10.1186/2047-783X-17-30
    CrossrefPubMedGoogle Scholar
  • 13 Jüngst C, Stadlbauer V, Reichert MC. et al. NOD2 gene variants confer risk for secondary sclerosing cholangitis in critically ill patients. Sci Rep 2017; 7: 7026 . doi:10.1038/s41598-017-06268-y
    CrossrefPubMedGoogle Scholar
  • 14 Deltenre P, Valla DC. Ischemic cholangiopathy. J Hepatol 2006; 44: 806-817
    CrossrefPubMedGoogle Scholar
  • 15 Trauner M, Fickert P, Wagner M. MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. Semin Liver Dis 2007; 27: 77-98
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 16 Beuers U, Hohenester S, de Buy Wenniger LJ. et al. The biliary HCO(3)(-) umbrella: a unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies. Hepatology 2010; 52: 1489-1496 . doi:10.1002/hep.23810
    CrossrefPubMedGoogle Scholar
  • 17 Evseenko DA, Paxton JW, Keelan JA. Independent regulation of apical and basolateral drug transporter expression and function in placental trophoblasts by cytokines, steroids, and growth factors. Drug Metab Dispos 2007; 35: 595-601
    CrossrefPubMedGoogle Scholar
  • 18 Verma S, Kaplowitz N. Diagnosis, management and prevention of drug-induced liver injury. Gut 2009; 58: 1555-1564
    CrossrefPubMedGoogle Scholar
  • 19 Koch A, Streetz K, Tischendorf J. et al. Leberwerterhöhung auf der Intensivstation. Med Klin Intensivmed Notfmed 2013; 108: 599-608 . doi:10.1007/s00063-013-0287-2
    CrossrefPubMedGoogle Scholar
  • 20 Fuchs M, Sanyal AJ. Sepsis and cholestasis. Clin Liver Dis 2008; 12: 151-172, ix . doi:10.1016/j.cld.2007.11.002
    CrossrefPubMedGoogle Scholar
  • 21 Voigtländer T, Leuchs E, Vonberg RP. et al. Microbiological analysis of bile and its impact in critically ill patients with secondary sclerosing cholangitis. J Infect 2015; 70: 483-490 . doi:10.1016/j.jinf.2015.01.013
    CrossrefPubMedGoogle Scholar
  • 22 Voigtländer T, Leuchs E, Vonberg RP. et al. Microbiological analysis of bile and its impact in critically ill patients with secondary sclerosing cholangitis. J Infect 2015; 70: 483-490 . doi:10.1016/j.jinf.2015.01.013
    CrossrefPubMedGoogle Scholar
  • 23 Kirchner GI, Rümmele P. Update on Sclerosing Cholangitis in Critically Ill Patients. Viszeralmedizin 2015; 31: 178-184
    PubMedGoogle Scholar
  • 24 Kulaksiz H, Heuberger D, Engler S. et al. Poor outcome in progressive sclerosing cholangitis after septic shock. Endoscopy 2008; 40: 214-218 . doi:10.1055/s-2007-967024
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 25 Kirchner GI, Scherer MN, Obed A. et al. Outcome of patients with ischemic-like cholangiopathy with secondary sclerosing cholangitis after liver transplantation. Scand J Gastroenterol 2011; 46: 471-478 . doi:10.3109/00365521.2010.537683
    CrossrefPubMedGoogle Scholar
  • 26 Voigtländer T, Negm AA, Schneider AS. et al. Secondary sclerosing cholangitis in critically ill patients: model of end-stage liver disease score and renal function predict outcome. Endoscopy 2012; 44: 1055-1058 . doi:10.1055/s-0032-1325733
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 27 Gossard AA, Angulo P, Lindor KD. Secondary sclerosing cholangitis: a comparison to primary sclerosing cholangitis. Am J Gastroenterol 2005; 100: 1330-1333
    CrossrefPubMedGoogle Scholar
  • 28 Weiss KE, Willmann JK, Jeffrey RB. et al. Secondary sclerosing cholangitis in a critically ill patient. Quant Imaging Med Surg 2016; 6: 224-228
    CrossrefPubMedGoogle Scholar
  • 29 Kwon ON, Cho SH, Park CK. et al. Biliary cast formation with sclerosing cholangitis in critically ill patient: case report and literature review. Korean J Radiol 2012; 13: 358-362
    CrossrefPubMedGoogle Scholar
  • 30 Esposito I, Kubisova A, Stiehl A. et al. Secondary sclerosing cholangitis after intensive care unit treatment: clues to the histopathological differenzial diagnosis. Virchows Arch 2008; 453: 339-345 . doi:10.1007/s00428-008-0654-1
    CrossrefPubMedGoogle Scholar
  • 31 Ponsioen CY, Arnelo U, Bergquist A. et al. No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis. Gastroenterology 2018; 155: 752-759.e5 . doi:10.1053/j.gastro.2018.05.034
    CrossrefPubMedGoogle Scholar
  • 32 Visseren T, Darwish Murad S. Recurrence of primary sclerosing cholangitis, primary biliary cholangitis and auto-immune hepatitis after liver transplantation. Best Pract Res Clin Gastroenterol 2017; 31: 187-198 . doi:10.1016/j.bpg.2017.04.004
    CrossrefPubMedGoogle Scholar
  • 33 Schielke A, Scatton O, Boelle PY. et al. Ischemic-type biliary lesions: A leading indication of liver retransplantation with excellent results. Clin Res Hepatol Gastroenterol 2019; 43: 131-139 . doi:10.1016/j.clinre.2017.11.005
    CrossrefPubMedGoogle Scholar