Download PDF
Semin Liver Dis 2016; 36(02): 109-116
DOI: 10.1055/s-0036-1583202
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acute-on-Chronic Liver Failure: Definition, Diagnosis, and Clinical Characteristics

Vicente Arroyo
1   Institut de Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain
2   European Foundation for the Study of Chronic Liver Failure (EF-CLIF) and European Association for the Study of the Liver-Chronic Liver Failure (EASL-CLIF) Consortium, Barcelona, Spain
,
Rajiv Jalan
2   European Foundation for the Study of Chronic Liver Failure (EF-CLIF) and European Association for the Study of the Liver-Chronic Liver Failure (EASL-CLIF) Consortium, Barcelona, Spain
3   Liver Failure Group, Institute for Liver and Digestive Health, University College London, Royal Free Hospital, London, United Kingdom
› Author Affiliations
Further Information

Publication History

Publication Date:
12 May 2016 (online)

    Permissions and Reprints

Abstract

Acute-on-chronic liver failure (ACLF) is a recently recognized syndrome in cirrhosis characterized by acute decompensation (AD), organ failure(s), and high short-term mortality. Organ failure(s) is defined by the Chronic Liver Failure-Sequential Organ Failure (CLIF-SOFA) score or by its simplified version Chronic Liver Failure-Organ Failure Assessment (CLIF-OF) score. They include six types of organ failure: liver, renal, coagulation, cerebral, respiratory, and circulatory. One third of patients hospitalized with AD present with ACLF at admission or develop ACLF during hospitalization. Acute-on-chronic liver failure frequently occurs in a closed relationship to a precipitating event. According to the number of organ failures, ACLF is graded into three stages: ACLF-1 = single renal failure or single nonrenal organ failure if associated with renal dysfunction and/or cerebral dysfunction; ACLF-2 = two organ failures; and ACLF-3 = three to six organ failures, with increasing 28-day mortality rate (from 23%–74%). Acute-on-chronic liver failure may develop at any phase during the clinical course of the disease. Patients without prior AD develop a severe form of ACLF.

Keywords

decompensated cirrhosis - organ failure - acute-on-chronic liver failure
 

  • References

  • 1 Ginés P, Quintero E, Arroyo V , et al. Compensated cirrhosis: natural history and prognostic factors. Hepatology 1987; 7 (1) 122-128
    CrossrefPubMedGoogle Scholar
  • 2 Schrier RW, Arroyo V, Bernardi M, Epstein M, Henriksen JH, Rodés J. Peripheral arterial vasodilation hypothesis: a proposal for the initiation of renal sodium and water retention in cirrhosis. Hepatology 1988; 8 (5) 1151-1157
    CrossrefPubMedGoogle Scholar
  • 3 Arroyo V, Ginès P, Gerbes AL , et al. Definition and diagnostic criteria of refractory ascites and hepatorenal syndrome in cirrhosis. Hepatology 1996; 23: 164-176
    CrossrefPubMedGoogle Scholar
  • 4 Ruiz-del-Arbol L, Monescillo A, Arocena C , et al. Circulatory function and hepatorenal syndrome in cirrhosis. Hepatology 2005; 42 (2) 439-447
    CrossrefPubMedGoogle Scholar
  • 5 Fede G, Spadaro L, Tomaselli T , et al. Adrenocortical dysfunction in liver disease: a systematic review. Hepatology 2012; 55 (4) 1282-1291
    CrossrefPubMedGoogle Scholar
  • 6 Grace JA, Angus PW. Hepatopulmonary syndrome: update on recent advances in pathophysiology, investigation, and treatment. J Gastroenterol Hepatol 2013; 28 (2) 213-219
    CrossrefPubMedGoogle Scholar
  • 7 Vilstrup H, Amodio P, Bajaj J , et al. Hepatic encephalopathy in chronic liver disease: 2014 Practice Guideline by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver. J Hepatol 2014; 60: 715-735
    PubMed
  • 8 Qin N, Yang F, Li A , et al. Alterations of the human gut microbiome in liver cirrhosis. Nature 2014; 513 (7516) 59-64
    CrossrefPubMedGoogle Scholar
  • 9 Wiest R, Lawson M, Geuking M. Pathological bacterial translocation in liver cirrhosis. J Hepatol 2014; 60 (1) 197-209
    CrossrefPubMedGoogle Scholar
  • 10 Bernardi M, Moreau R, Angeli P, Schnabl B, Arroyo V. Mechanisms of decompensation and organ failure in cirrhosis: from peripheral arterial vasodilation to systemic inflammation hypothesis. J Hepatol 2015; 63 (5) 1272-1284
    CrossrefPubMedGoogle Scholar
  • 11 Jalan R, Williams R. Acute-on-chronic liver failure: pathophysiological basis of therapeutic options. Blood Purif 2002; 20 (3) 252-261
    CrossrefPubMedGoogle Scholar
  • 12 Sarin SK, Kumar A, Almeida JA , et al. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL). Hepatol Int 2009; 3 (1) 269-282
    CrossrefPubMedGoogle Scholar
  • 13 Sarin SK, Kedarisetty CK, Abbas Z , et al; APASL ACLF Working Party. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL) 2014. Hepatol Int 2014; 8 (4) 453-471
    CrossrefPubMedGoogle Scholar
  • 14 Bajaj JS, O'Leary JG, Reddy KR , et al; North American Consortium for the Study of End-Stage Liver Disease Nacseld. Survival in infection-related acute-on-chronic liver failure is defined by extrahepatic organ failures. Hepatology 2014; 60 (1) 250-256
    CrossrefPubMedGoogle Scholar
  • 15 Jalan R, Gines P, Olson JC , et al. Acute-on chronic liver failure. J Hepatol 2012; 57 (6) 1336-1348
    CrossrefPubMedGoogle Scholar
  • 16 Moreau R, Jalan R, Gines P , et al; CANONIC Study Investigators of the EASL–CLIF Consortium. Acute-on-chronic liver failure is a distinct syndrome that develops in patients with acute decompensation of cirrhosis. Gastroenterology 2013; 144 (7) 1426-1437 , 1437.e1–1437.e9
    CrossrefPubMedGoogle Scholar
  • 17 Arroyo V, Moreau R, Jalan R, Ginès P ; EASL-CLIF Consortium CANONIC Study. Acute-on-chronic liver failure: a new syndrome that will re-classify cirrhosis. J Hepatol 2015; 62 (1, Suppl): S131-S143
    CrossrefPubMedGoogle Scholar
  • 18 Vincent JL, Moreno R, Takala J , et al. The SOFA (Sepsis-related Organ Failure Assessment) score to describe organ dysfunction/failure. Intensive Care Med 1996; 22 (7) 707-710
    CrossrefPubMedGoogle Scholar
  • 19 Jalan R, Saliba F, Pavesi M , et al; CANONIC study investigators of the EASL-CLIF Consortium. Development and validation of a prognostic score to predict mortality in patients with acute-on-chronic liver failure. J Hepatol 2014; 61 (5) 1038-1047
    CrossrefPubMedGoogle Scholar
  • 20 McPhail MJ, Shawcross DL, Abeles RD , et al. Increased survival for patients with cirrhosis and organ failure in liver intensive care and validation of the Chronic Liver Failure-Sequential Organ Failure Scoring System. Clin Gastroenterol Hepatol 2015; 13 (7) 1353-1360.e8
    CrossrefPubMedGoogle Scholar
  • 21 Dhiman RK, Agrawal S, Gupta T, Duseja A, Chawla Y. Chronic Liver Failure-Sequential Organ Failure Assessment is better than the Asia-Pacific Association for the Study of Liver criteria for defining acute-on-chronic liver failure and predicting outcome. World J Gastroenterol 2014; 20 (40) 14934-14941
    CrossrefPubMedGoogle Scholar
  • 22 Silva PE, Fayad L, Lazzarotto C , et al. Single-centre validation of the EASL-CLIF consortium definition of acute-on-chronic liver failure and CLIF-SOFA for prediction of mortality in cirrhosis. Liver Int 2015; 35 (5) 1516-1523
    CrossrefPubMedGoogle Scholar
  • 23 Lee M, Lee JH, Oh S , et al. CLIF-SOFA scoring system accurately predicts short-term mortality in acutely decompensated patients with alcoholic cirrhosis: a retrospective analysis. Liver Int 2015; 35 (1) 46-57
    CrossrefPubMedGoogle Scholar
  • 24 Li H, Pavesi M, Zeng B , et al. “Eastern” type of acute on chronic liver failure (ACLF) is similar in pathophysiologic, diagnostic and prognostic criteria to the “Western” type: a comparison between Chinese hospitalized patients with hepatitis B with CANONIC data. Hepatology 2014; 60: 480A-481A
    PubMedGoogle Scholar