Cirrhosis of the Liver

Background

• Cirrhosis of the liver is end-stage liver disease characterized by pathologic fibrosis and regenerative nodules with resultant liver dysfunction.
• Most common causes of cirrhosis in the Western world are alcohol misuse, nonalcoholic fatty liver disease, and viral hepatitis.
  • Increased alcohol consumption.
  • Hepatitis C most common viral cause of cirrhosis in US.
  • Hepatitis B most common viral cause of cirrhosis worldwide.

Evaluation

• Presentation:
  • Patient may be asymptomatic for many years.
  • Patient often presents for complications such as ascites, anasarca, variceal bleeding, jaundice, fatigue, and encephalopathy.
  • Other important complications include hepatocellular carcinoma, cholangiocarcinoma, coagulopathy, hepatorenal syndrome, hepatopulmonary syndrome, and portal pulmonary hypertension.
  • Clinical history may reveal alcohol misuse, history of obesity, bariatric surgery, blood product transfusion, IV drug use, risky sexual behavior, congenital heart disease, and family history of autoimmune or hepatic disease.
• Diagnosis:
  • Lok index, which utilizes platelet count, aspartate transaminase (AST), alanine transaminase (ALT), and INR, has a > 90% sensitivity for detecting cirrhosis. See DynaMed calculator for Lok index.
  • Bonacini cirrhosis discriminant score, which utilizes platelet count, ALT:AST ratio, and INR, has a > 90% specificity for a score > 7. See DynaMed calculator for Bonacini cirrhosis discriminant score.
  • Noninvasive testing with transient elastography, magnetic resonance elastrography, or acoustic radiation force impulse imaging is recommended (Strong recommendation).
  • Hepatic, renal, and coagulation function should be assessed along with a complete blood count when cirrhosis is suspected.
  • Blood tests for determining specific etiology of cirrhosis include hepatitis B and C serology, AST:ALT ratio, alpha-1 antitrypsin levels with phenotyping, antinuclear antibody, antismooth muscle titer, antimitochondrial antibodies, serum ferritin and transferrin saturation levels, hemochromatosis gene (HFE) testing (if iron saturation is greater than 45%), serum copper and ceruloplasmin, and 24-hour urine copper.
  • Liver biopsy should be considered in patients when the diagnosis is in question and knowledge of etiology may alter management and prognostic information may guide subsequent treatment (Weak recommendation).

Management

• Treat the underlying cause of the cirrhosis.
• If not immune, vaccinate with hepatitis A vaccine, hepatitis B vaccine, 23-valent pneumococcal vaccine.
• Screen patients for esophageal or gastric varices with endoscopy (Strong recommendation) and give primary prophylaxis (beta blockers, if tolerated) for variceal bleeding if there are large varices (> 5 mm) or high-risk features (red wale marks on varices or Child-Pugh Class B or C) (Strong recommendation).
• Screen for hepatocellular carcinoma with liver ultrasound and alpha fetoprotein measurement every 6 months (Strong recommendation).
• For patients with primary sclerosing cholangitis (PSC), screen for cholangiocarcinoma with liver ultrasound, and perform colonoscopy every 1-2 years if patient also has inflammatory bowel disease (Strong recommendation).
• Antibiotic prophylaxis for patients with cirrhosis and ascites without gastrointestinal bleeding may reduce mortality and rate of spontaneous bacterial peritonitis, but evidence may not be sufficient to support routine use.
• Antibiotic prophylaxis for patients with cirrhosis and gastrointestinal bleeding is recommended (Strong recommendation).
• Assess appropriateness of liver transplantation (Child-Pugh score > 7 or first major complication [ascites, variceal bleeding, or hepatic encephalopathy]).
• Moderate sodium-restricted diet (no added salt or preprepared meals) is recommended for patients with ascites (Strong recommendation).
• Free water restriction (< 1 L/day) is recommended for patients with hypervolemic hyponatremia (Strong recommendation).
• Model for End-Stage Liver Disease (MELD) score and Child-Pugh Score is used to predict 3-month and 1-year mortality respectively in patients with cirrhosis.