Acute-on-chronic liver failure: A new syndrome that will re-classify cirrhosis

• Published in: Journal of hepatology Vol. 62; no. 1; pp. S131 - S143
• Main Authors: Arroyo, Vicente, Moreau, Richard, Jalan, Rajiv, Ginès, Pere
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.04.2015
• Subjects: Acute decompensation of cirrhosis; Acute-on-chronic liver failure; Acute-On-Chronic Liver Failure - diagnosis; Acute-On-Chronic Liver Failure - etiology; Acute-On-Chronic Liver Failure - therapy; Artificial liver support systems; Danger/damage-associated molecular patterns (DAMPs); Gastroenterology and Hepatology; Hepatitis B - complications; Humans; Inflammation - complications; Liver Cirrhosis - classification; Organ failure; Pathogen-associated molecular patterns (PAMPs); Plasma exchange; Systemic inflammation; Toll-Like Receptors - physiology; norepinephrine; ACLF; FIO 2; CLIF-C ACLFs; C-Reactive Protein; hepatitis B virus; Child-Pugh score; CLIF Consortium ACLF score; Peripheral Blood Mononuclear Cells; Danger/damage-associated molecular patterns (DAMPs); MELD-Nas; PAMPs; Molecular Adsorbent Recirculating System; HBV; Sequential Organ Failure Assessment; Pattern-recognition Receptors; Acute Decompensation; AD; CPs; Artificial liver support systems; epinephrine; Nitric Oxide; ER; partial pressure of arterial oxygen; DAMPS; International Normalized Ratio; CLIF-C ADs; SOFA; Pathogen-associated molecular patterns (PAMPs); Acute-on-Chronic Liver Failure; fraction of inspired oxygen; NE; MELD-Sodium score; MAP; Acute decompensation of cirrhosis; MELDs; Systemic inflammation; CANONIC Study; CRP; Model of End-Stage Liver Disease; CLIF-SOFAs; NO; SpO 2; EASL-CLIF Acute-on-Chronic Liver Failure Study; E; PRRs; CLIF Consortium Organ Failure score; Endoplasmic Reticulum; INR; Lipopolysacharide; LPS; Chronic Liver Failure; Danger/Damage-Associated Molecular Patterns; Plasma exchange; EASL; hepatic encephalopathy; CLIF; PMBC; Pathogen-Associated Molecular Patters; European Association for the Study of the Liver; PaO 2; CLIF-C OFs; CLIF Consortium AD score; mean arterial pressure; CLIF-Sequential Organ Failure Assessment score; Toll-like Receptors; Organ failure; MARS; pulse oximetric saturation; HE; TLRs; Acute-on-chronic liver failure; FIO2; SpO2; PaO2
• ISSN: 0168-8278; 1600-0641
• DOI: 10.1016/j.jhep.2014.11.045

Summary Acute-on-chronic liver failure (ACLF) is a recently recognized syndrome characterized by acute decompensation (AD) of cirrhosis and organ/system failure(s) (organ failure: liver, kidney, brain, coagulation, circulation and/or respiration) and extremely poor survival (28-day mortality rate 30–40%). ACLF occurs in relatively young patients. It is especially frequent in alcoholic- and untreated hepatitis B associated-cirrhosis, in addition it is related to bacterial infections and active alcoholism, although in 40% of cases no precipitating event can be identified. It may develop at any time during the course of the disease in the patient (from compensated to long-standing cirrhosis). The development of ACLF occurs in the setting of a systemic inflammation, the severity of which correlates with the number of organ failures and mortality. Systemic inflammation may cause ACLF through complex mechanisms including an exaggerated inflammatory response and systemic oxidative stress to pathogen- or danger/damage-associated molecular patterns (immunopathology) and/or alteration of tissue homeostasis to inflammation caused either by the pathogen itself or through a dysfunction of tissue tolerance. A scoring system composed of three scores (CLIF-C OFs, CLIF-C AD, and CLIF-C ACLFs) specifically designed for patients with AD, with and without ACLF, allows a step-wise algorithm for a rational indication of therapy. The management of ACLF should be carried out in enhanced or intensive care units. Current therapeutic measures comprise the treatment for associated complications, organ failures support and liver transplantation.