What is decompensated cirrhosis?

Decompensated Cirrhosis

Decompensated cirrhosis is defined as the stage of liver cirrhosis marked by the development of overt clinical signs including ascites, variceal hemorrhage, hepatic encephalopathy, and/or jaundice, with a median survival of approximately 2 years. 1

Key Features and Definition

Decompensated cirrhosis represents a critical turning point in the progression of liver disease, characterized by:

• The transition from compensated cirrhosis (largely asymptomatic with preserved liver function) to decompensated cirrhosis
• A dramatic decline in median survival from 10-12 years in compensated cirrhosis to only 1-2 years after decompensation 1, 2
• Development of one or more clinical complications that signal liver failure and portal hypertension

Pathophysiology

Decompensation is primarily driven by:

• Progressive portal hypertension (HVPG >10 mmHg)
• Worsening liver function
• Systemic inflammation
• Vasodilatory-hyperdynamic circulatory state
• Decreased effective arterial blood volume 1
• Systemic inflammatory state (the "systemic inflammation hypothesis") 3

Clinical Manifestations

The four major complications that define decompensated cirrhosis include:

1. Ascites: Accumulation of fluid in the peritoneal cavity

   • Often the first sign of decompensation
   • Managed with sodium restriction, diuretics, and sometimes paracentesis 1

2. Variceal Hemorrhage: Bleeding from dilated veins in the esophagus or stomach

   • Requires prompt intervention with vasoactive drugs and endoscopic therapy
   • Associated with 20% 5-year mortality when occurring as an isolated complication
   • Mortality exceeds 80% when associated with other complications 1

3. Hepatic Encephalopathy: Neuropsychiatric manifestation ranging from subtle cognitive changes to coma

   • Often precipitated by infections, GI bleeding, or medications
   • Significantly impacts quality of life 1, 4

4. Jaundice: Yellowing of skin and sclera due to hyperbilirubinemia

   • Poor prognostic indicator
   • Often requires vasoactive drugs (terlipressin) and albumin 1

Clinical Course and Prognosis

Decompensation can occur through two distinct pathways:

1. Non-acute pathway: Slow development of ascites, mild hepatic encephalopathy, or jaundice not requiring hospitalization

   • Most frequent pathway of first decompensation 5

2. Acute pathway: Rapid onset of complications requiring hospitalization

   • Includes acute-on-chronic liver failure
   • Often represents further decompensation in already decompensated patients 5

The prognosis worsens with:

• Multiple decompensating events occurring simultaneously
• Recurrent episodes of decompensation
• Development of complications like hepatorenal syndrome

Management Approach

Management focuses on:

1. Treating the underlying cause of cirrhosis when possible (e.g., antiviral therapy for HBV-related cirrhosis) 1

2. Managing specific complications:

   • Ascites: Sodium restriction, diuretics, paracentesis, consideration of TIPS
   • Variceal hemorrhage: Vasoactive drugs, endoscopic therapy, pre-emptive TIPS for high-risk patients
   • Hepatic encephalopathy: Lactulose and rifaximin
   • Prevention of spontaneous bacterial peritonitis 1, 4

3. Preventing further decompensation:

   • Non-selective beta-blockers to lower portal hypertension
   • Albumin administration for specific indications
   • Addressing cofactors (obesity, diabetes, dyslipidemia, alcoholism) 1, 2

4. Evaluation for liver transplantation, especially for patients with clinical decompensation despite low MELD scores 1

Common Pitfalls in Management

• Using interferon-α in decompensated cirrhosis (contraindicated due to risk of serious complications) 1
• Failing to monitor for development of hepatocellular carcinoma
• Delaying referral for liver transplantation evaluation
• Not recognizing acute kidney injury early, which can lead to hepatorenal syndrome
• Inadequate prophylaxis against spontaneous bacterial peritonitis in high-risk patients

Patients with decompensated cirrhosis should be managed at centers with expertise in liver disease, as liver transplantation remains the definitive treatment for appropriate candidates 1.