Abstract
The etiology of the chronic liver disease (CLD) in patients with acute on chronic liver failure (ACLF) may vary from region to region. The major cause of underlying CLD is viral (hepatitis B and C) in the East, while it is alcohol related in the West and in some parts of the Indian subcontinent. Autoimmune liver disease and Wilson’s disease are the major underlying etiologies in the pediatric age group. The patients with CLD without cirrhosis should be included when defining ACLF. Non-alcoholic fatty liver disease related chronic liver insult in patients with known risk factors for progressive disease should be taken as a chronic liver disease in the setting of ACLF, whereas fatty liver with normal aminotransferases in low risk patients should not. The patients with CLD and previous decompensation should be excluded. Diagnosis of chronic liver disease in the setting of ACLF is made by history, physical examination and previously available or recent laboratory, endoscopic or radiological investigations. A liver biopsy through the transjugular route may help in cases where the presence of underlying CLD or its cause is not clear. The need of liver biopsy in ACLF should, however, be individualized. Standardization of liver biopsy assessment is essential for a uniform approach to the diagnosis and treatment of CLD and acute insult. Tools to measure liver stiffness may aid in identifying patients with advanced fibrosis. Studies are needed to validate the performance of these tests in the setting of ACLF.
Similar content being viewed by others
References
Sarin SK, Kumar A, Almeida JA, Chawla YK, Fan ST, Garg H, et al. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific Association for the study of the liver (APASL). Hepatol Int 2009;3:269–282
Bajaj JS. Defining acute on chronic liver failure: will East and West ever meet? Gastroenterology 2013;144:1337–1339
Sarin SK, Kedarisetty CK, Abbas Z, Amarapurkar D, Bihari C, Chan AC, et al. Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL) 2014. Hepatol Int 2014;8:453–471
Hamid SS, Atiq M, Shehzad F, Yasmeen A, Nissa T, Salam A, et al. Hepatitis E virus superinfection in patients with chronic liver disease. Hepatology 2002;36:474–478
Choi JY, Bae SH, Yoon SK, Cho SH, Yang JM, Han JY, et al. Preconditioning by extracorporeal liver support (MARS) of patients with cirrhosis and severe liver failure evaluated for living donor liver transplantation—a pilot study. Liver Int 2005;25:740–745
Du WB, Li LJ, Huang JR, Yang Q, Liu XL, Li J, et al. Effects of artificial liver support system on patients with acute or chronic liver failure. Transplant Proc 2005;37:4359–4364
Wagholikar GD, Lee KH, Pandey D, Leong SO, Singh R, Tan KC. Pre-transplant optimization by molecular adsorbent recirculating system in patients with severely decompensated chronic liver disease. Indian J Gastroenterol 2007;26:110–112
Wai CT, Lim SG, Aung MO, Lee YM, Sutedja DS, Dan YY, et al. MARS: a futile tool in centres without active liver transplant support. Liver Int 2007;27:69–75
Acharya SK, Sharma PK, Singh R, Mohanty SK, Madan K, Jha JK, et al. Hepatitis E virus (HEV) infection in patients with cirrhosis is associated with rapid decompensation and death. J Hepatol 2007;46:387–394
Kumar M, Sharma BC, Sarin SK. Hepatitis E virus as an etiology of acute exacerbation of previously unrecognized asymptomatic patients with hepatitis B virus-related chronic liver disease. J Gastroenterol Hepatol 2008;23:83–87
Krishna RY, Saraswat VA, Das K, Himanshu G, Yachha SK, Aggarwal R, et al. Clinical features and predictors of outcome in acute hepatitis A and hepatitis E virus hepatitis on cirrhosis. Liver Int 2009;29:392–398
Mahtab MA, Rahman S, Khan M, Karim MF. Hepatitis E virus is a leading cause of acute-on-chronic liver disease: experience from a tertiary centre in Bangladesh. Hepatobiliary Pancreat Dis Int 2009;8:50–52
Duseja A, Chawla YK, Dhiman RK, Kumar A, Choudhary N, Taneja S. Non-hepatic insults are common acute precipitants in patients with acute on chronic liver failure (ACLF). Dig Dis Sci 2010;55:3188–3192
Rastogi A, Kumar A, Sakhuja P, Bihari C, Gondal R, Hissar S, et al. Liver histology as predictor of outcome in patients with acute-on-chronic liver failure (ACLF). Virchows Arch 2011;459:121–127
El Sayed Zaki M, Othman W. Role of hepatitis E infection in acute on chronic liver failure in Egyptian patients. Liver Int 2011;31:1001–1005
Lal J, Thapa BR, Rawal P, Ratho RK, Singh K. Predictors of outcome in acute-on-chronic liver failure in children. Hepatol Int 2011;5:693–697
Garg H, Kumar A, Garg V, Sharma P, Sharma BC, Sarin SK. Clinical profile and predictors of mortality in patients of acute-on-chronic liver failure. Dig Liver Dis 2012;44:166–171
Jagadisan B, Srivastava A, Yachha SK, Poddar U. Acute on chronic liver disease in children from the developing world: recognition and prognosis. J Pediatr Gastroenterol Nutr 2012;54:77–82
Lin KH, Liu JW, Chen CL, Wang SH, Lin CC, Liu YW, et al. Impacts of pretransplant infections on clinical outcomes of patients with acute-on-chronic liver failure who received living-donor liver transplantation. PLoS One 2013;8(9):e72893
Xia Q, Dai X, Zhang Y, Guo Y, Xu X, Yang Q, et al. A modified MELD model for Chinese pre-ACLF and ACLF patients and it reveals poor prognosis in pre-ACLF patients. PLoS One 2013;8:e64379
Duan BW, Lu SC, Wang ML, Liu JN, Chi P, Lai W, et al. Liver transplantation in acute-on-chronic liver failure patients with high model for end-stage liver disease (MELD) scores: a single center experience of 100 consecutive cases. J Surg Res 2013;183:936–943
Xu X, Liu X, Ling Q, Wei Q, Liu Z, Xu X, et al. Artificial liver support system combined with liver transplantation in the treatment of patients with acute-on-chronic liver failure. PLoS One 2013;8(3):e58738
Duseja A, Choudhary NS, Gupta S, Dhiman RK, Chawla Y. APACHE II score is superior to SOFA, CTP and MELD in predicting the short-term mortality in patients with acute-on-chronic liver failure (ACLF). J Dig Dis 2013;14:484–490
Jha AK, Nijhawan S, Rai RR, Nepalia S, Jain P, Suchismita A. Etiology, clinical profile, and inhospital mortality of acute-on-chronic liver failure: a prospective study. Indian J Gastroenterol 2013;32:108–114
Garg H, Kumar A, Garg V, Kumar M, Kumar R, Sharma BC, et al. Hepatic and systemic hemodynamic derangements predict early mortality and recovery in patients with acute-on-chronic liver failure. J Gastroenterol Hepatol 2013;28:1361–1367
Kumar R, Krishnamoorthy TL, Tan HK, Lui HF, Chow WC. Change in model for end-stage liver disease score at two weeks, as an indicator of mortality or liver transplantation at 60 days in acute-on-chronic liver failure. Gastroenterol Rep (Oxf) 2014 (Epub ahead of print)
Khot AA, Somani P, Rathi P, Amarapurkar A. Prognostic factors in acute-on-chronic liver failure: a prospective study from western India. Indian J Gastroenterol 2014;33:119–124
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;20:14934–14941
Agrawal S, Duseja A, Gupta T, Dhiman RK, Chawla Y. Simple organ failure count versus canonic grading system for predicting mortality in acute on chronic liver failure. J Gastroenterol Hepatol 2015;30(3):575–581
Kedarisetty CK, Sarin SK, Anand L, Abbas Z, Amarapurkar DN, Bhardwaj A, et al. Liver failure determines the extra-hepatic organ failure and outcome in patients with acute-on-chronic liver failure: analysis of 1363 patients of AARC database. Hepatology 2014;60(Suppl. 1):556A
Shi Y, Zheng M, Ying Y, Wu W, Yang Q, Wu Y, et al. Prior decompensation is associated with delayed mortality in patients with acute-on-chronic liver failure. J Gastroenterol Hepatol 2015;30(4):712–718
Lee M, Lee JH, Oh S, Jang Y, Lee W, Lee HJ, 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
Mitzner SR, Stange J, Klammt S, Risler T, Erley CM, Bader BD, et al. Improvement of hepatorenal syndrome with extracorporeal albumin dialysis MARS: results of a prospective, randomized, controlled clinical trial. Liver Transplant 2000;6:277–286
Zauner C, Schneeweiss B, Schneider B, Madl C, Klos H, Kranz A, et al. Short-term prognosis in critically ill patients with liver cirrhosis: an evaluation of a new scoring system. Eur J Gastroenterol Hepatol 2000;12:517–522
Wehler M, Kokoska J, Reulbach U, Hahn EG, Strauss R. Short term prognosis in critically ill patients with cirrhosis assessed by prognostic scoring systems. Hepatology 2001;34:255–261
Heemann U, Treichel U, Loock J, Philipp T, Gerken G, Malago M, et al. Albumin dialysis in cirrhosis with superimposed acute liver injury: a prospective, controlled study. Hepatology 2002;36:949–958
Wasmuth HE, Kunz D, Yagmur E, Timmer-Stranghöner A, Vidacek D, Siewert E, et al. Patients with acute on chronic liver failure display “sepsis-like” immune paralysis. J Hepatol 2005;42:195–201
Cholongitas E, Senzolo M, Patch D, Kwong K, Nikolopoulou V, Leandro G, et al. Risk factors, sequential organ failure assessment and model for end-stage liver disease scores for predicting short term mortality in cirrhotic patients admitted to intensive care unit. Aliment Pharmacol Ther 2006;23:883–893
Katoonizadeh A, Laleman W, Verslype C, Wilmer A, Maleux G, Roskams T, et al. Early features of acute-on-chronic alcoholic liver failure: a prospective cohort study. Gut 2010;59:1561–1569
Moreau R, Jalan R, Gines P, Pavesi M, Angeli P, Cordoba J, et al. Acute-on-chronic liver failure is a distinct syndrome that develops in patients with acute decompensation of cirrhosis. Gastroenterology 2013;144:1426–1437
Angeli P, Rodríguez E, Piano S, Ariza X, Morando F, Solà E, et al. Acute kidney injury and acute-on-chronic liver failure classifications in prognosis assessment of patients with acute decompensation of cirrhosis. Gut 2014 (Epub ahead of print)
Jalan R, Saliba F, Pavesi M, Amoros A, Moreau R, Ginès P, 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;61:1038–1047
Silva PE, Fayad L, Lazzarotto C, Ronsoni MF, Bazzo ML, Colombo BS, 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 2014 (Epub ahead of print)
Bajaj JS, O’Leary JG, Reddy KR, Wong F, Biggins SW, Patton H, et al. Survival in infection-related acute-on-chronic liver failure is defined by extrahepatic organ failures. Hepatology 2014;60:250–256
Hytiroglou P, Snover DC, Alves V, Balabaud C, Bhathal PS, Bioulac-Sage P, et al. Beyond “cirrhosis”: a proposal from the International Liver Pathology Study Group. Am J Clin Pathol 2012;137:5–9
Kutami R, Girgrah N, Wanless IR, Sniderman K, Wong FS, Sherman M, et al. The Laennec grading system for assessment of hepatic fibrosis: validation by correlation with wedged hepatic vein pressure and clinical features (abstract). Hepatology 2000;32:407a
Wanless IR, Sweeney G, Dhillon AP, Guido M, Piga A, Galanello R, et al. Lack of progressive hepatic fibrosis during long-term therapy with deferiprone in subjects with transfusion-dependent beta-thalassemia. Blood 2002;100:1566–1569
Sen S, Davies NA, Mookerjee RP, Cheshire LM, Hodges SJ, Williams R, et al. Pathophysiological effects of albumin dialysis in acute-on-chronic liver failure: a randomized controlled study. Liver Transpl 2004;10:1109–1119
Gustot T. Multiple organ failure in sepsis: prognosis and role of systemic inflammatory response. Curr Opin Crit Care 2011;17:153–159
Jalan R, Gines P, Olson JC, Mookerjee RP, Moreau R, Garcia-Tsao G, et al. Acute-on chronic liver failure. J Hepatol 2012;57:1336–1348
Moreno RP, Metnitz B, Adler L, Hoechtl A, Bauer P, Metnitz PG, et al. Sepsis mortality prediction based on predisposition, infection and response. Intensiv Care Med 2008;34:496–504
Kamath PS, Wiesner RH, Malinchoc M, Kremers W, Therneau TM, Kosberg CL, et al. A model to predict survival in patients with end-stage liver disease. Hepatology 2001;33:464–470
Vincent JL, Moreno R, Takala J, Willatts S, De Mendonça A, Bruining H, et al. The SOFA (sepsis-related organ failure assessment) score to describe organ dysfunction/failure. Intensiv Care Med 1996;22:707–710
Wang S, Kamat A, Pergola P, Swamy A, Tio F, Cusi K. Metabolic factors in the development of hepatic steatosis and altered mitochondrial gene expression in vivo. Metabolism 2011;60:1090–1099
Fabbrini E, Magkos F, Mohammed BS, Pietka T, Abumrad NA, Patterson BW, et al. Intrahepatic fat, not visceral fat, is linked with metabolic complications of obesity. Proc Natl Acad Sci USA 2009;106:15430–15435
Pais R, Pascale A, Fedchuck L, Charlotte F, Poynard T, Ratziu V. Progression from isolated steatosis to steatohepatitis and fibrosis in nonalcoholic fatty liver disease. Clin Res Hepatol Gastroenterol 2011;35:23–28
Pais R, Charlotte F, Fedchuk L, Bedossa P, Lebray P, Poynard T, et al. A systematic review of follow-up biopsies reveals disease progression in patients with non-alcoholic fatty liver. J Hepatol 2013;59:550–556
Wong VW, Wong GL, Choi PC, Chan AW, Li MK, Chan HY, et al. Disease progression of non-alcoholic fatty liver disease: a prospective study with paired liver biopsies at 3 years. Gut 2010;59:969–974
Kotecha HL, Saraf N, Saigal S, Choudhary NS, Yadav A, Mohanka R, et al. NAFLD is the leading cause of donor rejection in living donor liver transplantation. J Clin Exp Hepatol 2013;3:S3
Urena MA, Ruiz-Delgado FC, Gonzalez EM, Segurola CL, Romero CJ, Garcia IG, et al. Assessing risk of the use of livers with macro and microsteatosis in a liver transplant program. Transpl Proc 1998;30:3288–3291
Starley BQ, Calcagno CJ, Harrison SA. Nonalcoholic fatty liver disease and hepatocellular carcinoma: a weighty connection. Hepatology 2010;51:1820–1832
Ratziu V, Charlotte F, Heurtier A, Gombert S, Giral P, Bruckert E, et al. Sampling variability of liver biopsy in nonalcoholic fatty liver disease. Gastroenterology 2005;28:1898–1906
Adams LA, Sanderson S, Lindor KD, Angulo P. The histological course of nonalcoholic fatty liver disease: a longitudinal study of 103 patients with sequential liver biopsies. J Hepatol 2005;42:132–138
Vernon G, Baranova A, Younossi ZM. Systematic review: the epidemiology and natural history of non-alcoholic fatty liver disease and non-alcoholic steatohepatitis in adults. Aliment Pharmacol Ther 2011;34:274–285
Adams LA, Lymp JF, St Sauver J, Sanderson SO, Lindor KD, Feldstein A, et al. The natural history of nonalcoholic fatty liver disease: a population-based cohort study. Gastroenterology 2005;129:113–121
Harrison SA, Torgerson S, Hayashi PH. The natural history of nonalcoholic fatty liver disease: a clinical histopathological study. Am J Gastroenterol 2003;98:2042–2047
Sakhuja P, Rastogi A, Gondal R, Garg H, Sarin SK. Acute on chronic liver failure-analysis of two distinct liver histological patterns [abstract]. J Hepatol 2008;48(Suppl. 2):S95
Colloredo G, Guido M, Sonzogni A, Leandro G. Impact of liver biopsy size on histological evaluation of chronic viral hepatitis: the smaller the sample, the milder the disease. J Hepatol 2003;39:239–244
Rousselet MC, Michalak S, Dupré F, Croué A, Bedossa P, Saint-André JP, et al. Sources of variability in histological scoring of chronic viral hepatitis. Hepatology 2005;41:257–264
Cholongitas E, Quaglia A, Samonakis D, Mela M, Patch D, Dhillon AP, et al. Transjugular liver biopsy in patients with diffuse liver disease: comparison of three cores with one or two cores for accurate histological interpretation. Liver Int 2007;27:646
Brunt EM. Liver biopsy interpretation for the gastroenterologist. Curr Gastroenterol Rep 2000;2:27–32
Ishak K, Baptista A, Bianchi L, Callea F, De Groote J, Gudat F, et al. Histological grading and staging of chronic hepatitis. J Hepatol 1995;22:696–699
Myers RP, Pomier-Layrargues G, Kirsch R, Pollett A, Duarte-Rojo A, Wong D, et al. Feasibility and diagnostic performance of the FibroScan XL probe for liver stiffness measurement in overweight and obese patients. Hepatology 2012;55:199–208
Fraquelli M, Rigamonti C, Casazza G, Donato MF, Ronchi G, Conte D, et al. Etiology-related determinants of liver stiffness values in chronic viral hepatitis B or C. J Hepatol 2011;54:621–628
Crespo G, Fernández-Varo G, Mariño Z, Casals G, Miquel R, Martínez SM, et al. ARFI, FibroScan, ELF, and their combinations in the assessment of liver fibrosis: a prospective study. J Hepatol 2012;57:281–287
Karlas TF, Pfrepper C, Rosendahl J, Benckert C, Wittekind C, Jonas S, et al. Acoustic radiation force impulse (ARFI) elastography in acute liver failure: necrosis mimics cirrhosis. Z Gastroenterol 2011;49:443–448
Godfrey EM, Patterson AJ, Priest AN, Davies SE, Joubert I, Krishnan AS, et al. A comparison of MR elastography and 31P MR spectroscopy with histological staging of liver fibrosis. Eur Radiol 2012;22:2790–2797
Kim D, Kim WR, Talwalkar JA, Kim HJ, Ehman RL. Advanced fibrosis in nonalcoholic fatty liver disease: noninvasive assessment with MR elastography. Radiology 2013;268:411–419
Talwalkar JA, Yin M, Fidler JL, Sanderson SO, Kamath PS, Ehman RL. Magnetic resonance imaging of hepatic fibrosis: emerging clinical applications. Hepatology 2008;47:332–342
Wai CT, Greenson JK, Fontana RJ, Kalbfleisch JD, Marrero JA, Conjeevaram HS, et al. A simple noninvasive index can predict both significant fibrosis and cirrhosis in patients with chronic hepatitis C. Hepatology 2003;38:518–526
Castéra L, Vergniol J, Foucher J, Le Bail B, Chanteloup E, Haaser M, et al. Prospective comparison of transient elastography, Fibrotest, APRI, and liver biopsy for the assessment of fibrosis in chronic hepatitis C. Gastroenterology 2005;128:343–350
Becker L, Salameh W, Sferruzza A, Zhang K, Chen R, Malik R, et al. Validation of hepascore, compared with simple indices of fibrosis, in patients with chronic hepatitis C virus infection in United States. Clin Gastroenterol Hepatol 2009;7:696–701
Schwenzer NF, Springer F, Schraml C, Stefan N, Machann J, Schick F. Non-invasive assessment and quantification of liver steatosis by ultrasound, computed tomography and magnetic resonance. J Hepatol 2009;51:433–445
Chalasani N, Younossi Z, Lavine JE, Diehl AM, Brunt EM, Cusi K, et al. The diagnosis and management of non-alcoholic fatty liver disease: practice guideline by the American Association for the Study of Liver Diseases, American College of Gastroenterology, and the American Gastroenterological Association. Hepatology 2012;55:2005–2023
Ratziu V, Bellentani S, Cortez-Pinto H, Day C, Marchesini G. A position statement on NAFLD/NASH based on the EASL 2009 special conference. J Hepatol 2010;53:372–384
Loria P, Adinolfi LE, Bellentani S, Bugianesi E, Grieco A, Fargion S, et al. Practice guidelines for the diagnosis and management of nonalcoholic fatty liver disease. A decalogue from the Italian Association for the Study of the Liver (AISF) Expert Committee. Dig Liver Dis 2010;42:272–282
Angulo P, Hui JM, Marchesini G, Bugianesi E, George J, Farrell GC, et al. The NAFLD fibrosis score: a noninvasive system that identifies liver fibrosis in patients with NAFLD. Hepatology 2007;45:846–854
Guha IN, Parkes J, Roderick P, Chattopadhyay D, Cross R, Harris S, et al. Noninvasive markers of fibrosis in nonalcoholic fatty liver disease: validating the European Liver Fibrosis Panel and exploring simple markers. Hepatology 2008;47:455–460
Pagadala M, Zein CO, McCullough AJ. Predictors of steatohepatitis and advanced fibrosis in non-alcoholic fatty liver disease. Clin Liver Dis 2009;13:591–606
Compliance with ethical requirements and Conflict of interest
All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008 (5). The study protocol was approved by the National Liver Institute Institutional Review Board and the Ministry of Health IRB. Zaigham Abbas and Lubna Shazi declare that they have no conflict of interest.
Informed consent
Informed consent was obtained from all patients for being included in the study.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Abbas, Z., Shazi, L. Pattern and profile of chronic liver disease in acute on chronic liver failure. Hepatol Int 9, 366–372 (2015). https://doi.org/10.1007/s12072-015-9627-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12072-015-9627-z