What are the clinical features and management of decompensated liver disease?

Clinical Features and Management of Decompensated Liver Disease

Definition and Cardinal Clinical Features

Decompensated cirrhosis is defined by the presence of ascites, variceal hemorrhage, hepatic encephalopathy, or jaundice—any one of these complications marks the critical transition from compensated to decompensated disease. 1, 2

The Four Cardinal Manifestations:

Ascites:

• Most common first decompensating event, occurring in approximately 50% of patients within 10 years of cirrhosis diagnosis 2
• Presents as abdominal distension, weight gain, and peripheral edema 2
• Grade 2 or 3 ascites requires diagnostic paracentesis in all patients 3

Variceal Hemorrhage:

• Presents as hematemesis or melena 2
• Gastroesophageal varices increase from 30-40% in compensated cirrhosis to 85% in decompensated disease 2
• Constitutes 70% of all upper GI bleeding in portal hypertension and is the second most frequent decompensating event after ascites 1

Hepatic Encephalopathy:

• Ranges from subtle cognitive changes to coma 2
• Early signs include confusion, personality changes, sleep disturbances, and asterixis 2
• Simple screening tests like animal naming can detect minimal hepatic encephalopathy 3

Jaundice:

• Yellow discoloration of skin and sclera from elevated bilirubin 2
• Indicates progressive liver failure and worsening synthetic function 2

Additional Critical Complications:

Renal Dysfunction:

• Hepatorenal syndrome presents with progressive oliguria and rising creatinine 2
• Hyponatremia indicates advanced disease with 20% mortality at 1 year 2

Spontaneous Bacterial Peritonitis:

• Presents with fever, abdominal pain, and altered mental status 2
• Bacterial infections particularly accelerate disease progression in decompensated patients 2

Prognostic Impact

Median survival drops dramatically from >12 years in compensated cirrhosis to approximately 2 years after first decompensation. 2 Further decompensation with recurrent complications (refractory ascites, recurrent variceal hemorrhage, or recurrent hepatic encephalopathy) carries significantly worse prognosis 2.

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Immediate Management Priorities

Initial Stabilization and Assessment:

Airway, breathing, and circulation assessment with rapid volume replacement using crystalloids is crucial for hemodynamic stability. 3

Diagnostic paracentesis must be performed in all patients with new-onset grade 2 or 3 ascites, or those hospitalized for any cirrhosis complication. 3

All patients with decompensated cirrhosis should be managed in institutions capable of handling complications and referred immediately for transplant evaluation. 1, 3

Etiological Treatment:

Treating the underlying cause immediately is essential and may lead to recompensation, decreased risk of further decompensation, and improved survival. 1, 3, 4

For Hepatitis B-related decompensated cirrhosis:

• Start antiviral therapy immediately regardless of ALT levels or HBV DNA levels 3, 4
• Use entecavir 1 mg daily or tenofovir monotherapy as first-line agents due to potent antiviral efficacy and high genetic barrier to resistance 1, 3, 4
• Interferon-α is absolutely contraindicated in decompensated cirrhosis due to risk of precipitating liver failure and serious infections 1, 3
• Prolonged adequate HBV suppression can stabilize patients, improve Child-Turcotte-Pugh scores, and delay or avoid transplantation 1, 3

For Hepatitis C-related decompensated cirrhosis:

• Direct-acting antivirals improve liver function and portal hypertension 4

For alcohol-related cirrhosis:

• Alcohol cessation is associated with decreased risk of decompensation and increased survival 1

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Management of Specific Complications

Ascites Management:

First-line treatment combines moderate sodium restriction (80-120 mmol/day, equivalent to 4.6-6.9 g of salt) with spironolactone with or without furosemide. 3, 4

Avoid nephrotoxic medications, NSAIDs, and aminoglycosides during decompensation episodes. 3

Close monitoring of renal function is vital to detect hepatorenal syndrome early. 3

Variceal Hemorrhage Management:

Initiate vasoactive medications (terlipressin, somatostatin, or octreotide) immediately upon suspicion of variceal hemorrhage, even before endoscopic confirmation. 3, 4

Apply restrictive transfusion strategy with hemoglobin threshold of 7 g/dL and target of 7-9 g/dL to avoid increasing portal pressure. 3

Perform upper endoscopy within the first 12 hours of admission. 3

Endoscopic variceal ligation is the treatment of choice for esophageal varices. 3

Administer prophylactic antibiotics immediately and continue for up to 7 days (ceftriaxone 1g/day IV for patients with decompensated cirrhosis). 3

Initiate secondary prophylaxis with non-selective beta-blockers and/or endoscopic variceal ligation once bleeding is controlled. 3

Hepatic Encephalopathy Management:

Use lactulose or lactitol as first-line therapy to reduce mortality and prevent recurrent overt hepatic encephalopathy. 3, 4

Identify and treat precipitating factors including infection, bleeding, and medications. 3

Monitor continuously for changes in mental status. 3

Prevention Strategies:

Non-selective beta-blockers can prevent decompensation in patients with clinically significant portal hypertension, mainly by reducing the risk of ascites. 1, 5

Use beta-blockers with caution in patients with severe or refractory ascites. 3

Strategies targeting gut-liver axis abnormalities (rifaximin), improving circulatory function (long-term albumin administration), and decreasing inflammation (statins) show promise but require further validation. 1

Norfloxacin reduces the risk of spontaneous bacterial peritonitis and hepatorenal syndrome, though concerns exist regarding resistant bacteria. 1

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Critical Pitfalls to Avoid

Never delay antiviral therapy waiting for specific HBV DNA thresholds—any detectable level requires immediate treatment in decompensated cirrhosis. 3

Never use interferon under any circumstances in decompensated patients due to risk of precipitating liver failure and serious infections. 1, 3

Never overlook the need for immediate transplant evaluation—decompensation is itself an indication for referral regardless of MELD score. 3, 6

Patients with ascites should generally be considered for referral to a liver transplantation program as this represents a critical prognostic threshold. 3

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Multidisciplinary Approach and Follow-up

A multidisciplinary team approach involving hepatologists, radiologists, and surgeons dedicated to advanced chronic liver disease optimizes outcomes. 4

All patients with decompensated cirrhosis should be sent for hepatological evaluation for optimal management of portal hypertension complications. 4

MELD and MELD-Na scores guide transplant prioritization, though clinical decompensation with low MELD still warrants transplant consideration given the significant mortality reduction (~40%) with transplantation. 4, 6