Cirrhosis

Background

• End-stage hepatic fibrosis with hepatic dysfunction

Epidemiology

• 25% of Canadians have NAFLD

Etiology

• Infectious
  • Chronic hepatitis B
  • Chronic hepatitis C
  • Brucellosis, echinococcosis, syphilis
• Toxins and drugs
  • Alcohol
  • Medications: isoniazid, methotrexate
• Autoimmune/inflammatory
  • Autoimmune hepatitis (AIH)
  • Primary biliary cirrhosis (PBC)
  • Primary sclerosing cholangitis
  • Celiac disease
• Metabolic
  • NASH
  • Wilson disease
  • Hereditary hemochromatosis
  • α-1 antitrypsin deficiency
• Other
  • Right-sided heart failure
  • Veno-occlusive disease
  • Hereditary hemorrhagic telangiectasia
  • Polycystic liver disease
  • Granulomatous liver disease

JAMA Rational Clinical Exam

• Ascites (LR+ 7.2)
• Platelet count <160 (LR+ 6.3; LR- 0.29 if ≥160)
• Spider nevi (LR+ 4.3)
• Bonacini cirrhosis discriminant score >7 (LR+ 9.4)
• Lok index <0.2 (LR- 0.09)
• Absence of hepatomegaly (LR- 0.37)

Investigations

• Lab abnormalities in liver failure, in order:
  • Decrease in platelets
  • Increase in bilirubin
  • Decrease in albumin
  • Increase in INR
• Investigations for possible causes of cirrhosis include:
  • Liver ultrasound
  • Hepatitis B and C serologies
  • Ferritin and transferrin saturation, for hereditary hemochromatosis
  • Autoimmune serologies
    • ANA and anti-smooth muscle antibodies, for PSC and AIH
    • Anti-mitochondrial antibodies and anti-parietal cell antibodies, for PBC
    • Consider anti-LKM-1, for PSC
  • Quantitative immunoglobulins, for elevated IgM in PBC or hypergammaglobulinemia in AIH
  • Consider serum ceruloplasmin, for Wilson disease
  • Consider α-1 antitripsyin levels, for deficiency

Management

• Decompensated
  • Rule out spontaneous bacterial peritonitis with every episode
• Ascites
  • Furosemide 40 and spironolactone 100 (max 160/400)
  • Serial therapeutic paracentesis
    • Small-volume (<4-5L) does not need albumin
    • Large-volume needs 6-8 g of 25% albumin per litre removed
  • Avoid indwelling drain
  • TIPS can be considered but precipitates encephalopathy
• Prophylaxis/chronic
  • SBP: maybe?
  • Encephalopathy: no role for primary prophylaxis
  • Surveillance with upper endoscopy
  • Ultrasound q6mo for HCC

Preventative Care

• Avoid raw oysters (high risk of Vibrio vulnificans infection in cirrhosis)

Prognosis

• Median survival 1.6 years after admission for decompensated cirrhosis