Acute-on-chronic liver failure vs. traditional acute decompensation of cirrhosis

Abstract

Clinical vignette A 34-year-old woman with a history of chronic HCV-related cirrhosis was admitted to the hospital emergency department for an acute decompensation characterised by jaundice and ascites. A diagnosis of community-acquired spontaneous bacterial peritonitis complicated by bacteraemia with Escherichia coli was made. Despite prompt, adequate antibiotic treatment (ceftriaxone for 7 days), albumin infusion and adequate response (decrease of at least 25% of ascitic neutrophils at 48 h after the start of antibiotic), physicians observed a severe clinical deterioration with the development of neurological and respiratory failure. The patient was referred to the intensive care unit of University Hospital. At admission, the patient was mechanically ventilated with severe respiratory parameters (low tidal volume, positive end expiratory pressure [PEEP] of 15 cm H 2 O and fractional inspired oxygen [FiO 2 ] 50%, resulting in arterial oxygen partial pressure [PaO 2 ] of 61 mmHg, PaO 2 /FiO 2 ratio of 122), stage IV hepatic encephalopathy, type I hepatorenal syndrome that responded to terlipressin 4 mg per day (creatinine 1.1 mg/dl), international normalized ratio (INR) 3.58, and total bilirubin 22 mg/dl. Thoracic computed tomography showed slight bilateral pleural effusion and left basal condensation. A systematic microbiological screening including bronchoalveolar lavage, blood, urinary and ascitic cultures did not demonstrate any overt infection. After three days of intensive management, the clinical situation did not improve significantly (mechanical ventilation with PEEP 12 cmH 2 O, FiO 2 50%, PaO 2 65 mmHg and a PaO 2 /FiO 2 ratio of 130, stage IV hepatic encephalopathy, serum ammonia level of 376 lg/dl, INR 2.98, total bilirubin 30 mg/dl, creatinine 0.9 mg/dl under terlipressin 4 mg per day). Thus, orthotopic liver transplantation was considered the only long-term life-saving option for this patient. However, this candidate seemed to be too sick to receive an organ. This case prompts many clinical questions, including: I. Are there specificities in this acute decompensation of cirrhosis? Is the concept of ACLF relevant for this case? II. Which precipitating events and pathogenic mechanisms are responsible for ACLF? III. Do we have tools to predict outcomes and clinical courses for ACLF? IV. What are the current management strategies, supported by evidence, for patients with ACLF? V. Is salvage liver transplantation a reasonable therapeutic option for ACLF? What is the ideal timing for liver transplantation? VI. Are there therapeutic (including experimental) strategies to improve survival or to bridge the patient to liver transplantation?

Risk factors for progression to acute-on-chronic liver failure during severe acute exacerbation of chronic hepatitis B virus infection

“…In patients with chronic HBV infection, HD manifesting as ascites, esophagogastric variceal bleeding (EVB), or hepatic encephalopathy is a significant stage during progression from AE to liver failure[11,12]. Once progressed to HD, the liver function of patients with AE or SAE becomes more unstable and undergoes more rapid deterioration to ACLF following intrahepatic or extrahepatic insults[13,14]. Despite improved clinical management, the mortality of patients with ACLF ranges from 50%-80% without liver transplantation[15].…”

Therapeutic plasma exchange in acute on chronic liver failure

“…Although prospective evidence on ACLF and liver transplantation are still considered rather scarce, recent French data show high one‐year post transplant survival of 84% even in high grade ACLF patients 19 and centers from Asian countries have reported similar favorable outcomes in such patients following liver transplantation 20 . Hence, we believe that it is important to not generally exclude patients with high grade ACLF prematurely from the opportunity of transplantation 21 . However, our data suggest that if a negative decision against transplantation has been made in this kind of severe ACLF patients there might not be a place to continue TPE but rather to consider a best supportive care scenario.…”

Akut-auf-chronisches Leberversagen (ACLF)

“…Wenn ein ACLF vorliegt, lässt sich die individuelle Prognose des Patienten auf folgender Grundlage einschätzen: 1. dem initialen Grad des ACLF bei Diagnose, 2. dem weiteren klinischen Verlauf und 3. den spezifischen Prognose-Scores [5]. Der initiale Grad des ACLF wird je nach Anzahl der Organversagen in 3 Stufen eingeteilt: ACLF Grad 1-3.…”