Cirrhosis Demo



Type

Clinical features

Definition

  • Progressive hepatic fibrosis with distorted architecture and regenerative nodules

Etiologies

  • Hepatitis B and C
  • Alcoholic liver disease
  • Nonalcoholic liver disease
  • Hemochromatosis
  • Autoimmune hepatitis
  • Primary biliary cholangitis (PBC)
  • Primary sclerosing cholangitis (PSC)
  • Medications (eg, methotrexate)
  • Wilson disease
  • Celiac disease
  • Alpha-1 antitrypsin disease
  • Polycystic liver disease
  • Infections (eg, brucella)
  • Right sided congestive heart failure (Cardiac cirrhosis)
  • Polycystic liver disease
  • Veno-occlusive disease

Stages of liver disease

Type

Clinical Features

Possible Symptoms

  • Compensated
    • Can be asymptomatic
    • Anorexia
    • Weight loss
    • Weakness and/or fatigue
  • Decompensated
    • Pruritus
    • Jaundice
    • GI bleeding (hematemesis, melena, hematochezia)
    • Abdominal distention (ascites)
    • Confusion (hepatic encephalopathy)
    • Muscle cramps
    • Fatigue
    • Lower extremity edema
    • Weight changes
    • Diarrhea
    • Pruritus
    • Amenorrhea in women
    • Hypogonadism in men (eg, erectile dysfunction)

Possible physical exam findings

  • Skin
  • Head and neck
    • Icterus in eyes
    • Enlarged parotid gland
    • Fetor hepaticus (sweet pungent breath)
  • Chest
  • Abdomen
    • Hepatomegaly
      • Can also be small or normal size depending on stage of liver disease
    • Splenomegaly
    • Ascites
      • Abdominal distention, fluid wave, dullness to percussion
    • Caput Medusa
      • Portal hypertension causes dilation of periumbilical veins in shape of Medusa’s head
    • Cruveilhier-Baumgarten murmur
      • Venous hum heard best over the epigastric area due to collateral connections between portal vein and remnant of umbilical vein
  • Extremities
  • Others
    • Asterixis in hepatic encephalopathy
    • Testicular atrophy

Possible laboratory/ imaging findings

  • Laboratory findings suggesting cirrhosis
    • Elevated AST > ALT in 2:1 ratio as cirrhosis develops
    • Elevated bilirubin and GGT
    • Decreased albumin
    • Elevated PT/INR
    • Hyponatremia with disease progression
    • Anemia, leukopenia, and thrombocytopenia
  • Imaging findings suggesting cirrhosis
    • Ultrasound
      • Can show small and nodular liver, increased echogenicity, and irregular appearing areas
      • Evidence of portal hypertension, ascites, and portal vein thrombosis
    • Elastography
      • Increased stiffness of the tissue

Complications of cirrhosis

  • Ascites
  • Hepatic encephalopathy
  • Hepatocellular carcinoma
  • Portal hypertensive gastropathy
  • Portal vein thrombosis
  • Umbilical hernia
  • Variceal bleeding
    • Gastric, esophageal, splenic, rectal
  • Spontaneous bacterial peritonitis (SBP)
    • Positive ascites fluid for bacterial growth
    • Elevated ascitic WBC > 250 cells/mm3
  • Hepatorenal syndrome
    • Renal failure in patients with advanced cirrhosis
  • Hepatic hydrothorax
    • Pleural effusion (typically right sided) in cirrhosis without underlying cardiac or pulmonary disease
  • Hepatopulmonary syndrome
    • Liver disease
    • Increased alveolar-arterial gradient while breathing room air
    • Evidence for intrapulmonary vascular abnormalities (intrapulmonary vascular dilatations)
  • Porto-pulmonary hypertension
    • Pulmonary hypertension with portal hypertension

Type

Recommendations

Clinical clues

  • Stigmata of liver disease on physical examination
  • Laboratory studies suggesting cirrhosis
  • Development of complication of cirrhosis
  • Imaging suggesting cirrhosis

Liver biopsy

  • Gold standard for diagnosing cirrhosis
  • However, not necessary if clinical clues are consistent with likely cirrhosis

Determine etiology of cirrhosis

  • Alcohol history with AST/ALT > 2
  • Hepatitis B and C testing
  • Antimitochondrial antibodies for suspected PBC
  • ANA, ANCA, cholangiography for PSC
  • ANA, Anti-smooth muscle antibodies, ANCA for suspected autoimmune hepatitis
  • Transferrin, plasma ferritin, and gene testing for hemochromatosis
  • Serum ceruloplasmin for Wilson disease
  • Serum alpha-1 antitrypsin
  • RUQ ultrasound
  • Eventual liver biopsy

Type

Recommendations

General

  • Slow or reverse progression of liver disease
    • Abstain from alcohol use
    • Treatment of underlying etiology if known
    • Hepatitis A and B vaccines
    • Yearly influenza vaccination
    • Avoid hepatotoxins (eg, medications, supplements)
    • Adjust medications and dosages for hepatic clearance
  • Muscle cramps
    • Replete electrolytes if abnormal
    • Branched chain amino acids 4 g TID
    • Taurine 3 g/day
    • Vitamin E 200 mg TID
  • Umbilical hernia
    • Aggressive ascites management
    • Consider surgical repair for refractory patients
  • Elevated PT/INR or thrombocytopenia
    • Only treat with plasma for upcoming procedure or active bleeding
  • Liver transplant
    • Can be a definitive therapy depending on underlying etiology

Treatment of Ascites

  • General
    • Abstain from alcohol
    • Treat underlying cause of liver disease if known
    • Avoid medications that decrease blood pressure (eg, ARB, ACE)
    • Avoid NSAIDs
    • Avoid beta blockers in refractory ascites
    • Restrict sodium intake to 2 g/day
  • Diuretics
    • Spironolactone (Aldactone) 50-100 mg/day
      • Measure urine electrolytes to assess urinary sodium and potassium excretion
    • Add Furosemide (Lasix) 40 mg/day + Spironolactone (Aldactone) 100 mg/day
      • Measure urine electrolytes to assess response and adjust dosage
  • Refractory ascites
    • Large volume paracentesis
    • Transjugular intrahepatic portosystemic shunts (TIPS)
    • Liver transplantation

Routine screening/ prevention

  • Variceal bleeding
    • Periodic upper endoscopy for screening for varices
    • Propranolol daily in patients with varices
  • Hepatocellular carcinoma
    • RUQ ultrasound every 6 months
  • SBP
    • Treat other infections (eg, cellulitis) aggressively to prevent SBP
    • Prophylactic antibiotics during hospitalizations
  • Hepatic encephalopathy
    • Avoid triggers
      • Variceal bleeding
      • Infections
      • Sedatives
      • Hypokalemia
      • Hyponatremia
      • Medications
      • Constipation
      • Volume depletion
    • Low protein diet
  • Hepatorenal syndrome
    • Avoid nephrotoxic medications

Child-Pugh classification for severity/prognosis

  • Ascites
    • Absent (1 point)
    • Slight (2 points)
    • Moderate (3 points)
  • Bilirubin
    • < 2 mg/dL (1 point)
    • 2-3 mg/dL (2 points)
    • > 3 mg/dL (3 points)
  • Albumin
    • > 3.5 g/dL (1 point)
    • 2.8-3.5 g/dL (2 points)
    • < 2.8 g/dL (3 points)
  • INR
    • < 1.7 (1 point)
    • 1.7-2.3 (2 points)
    • > 2.3 (3 points)
  • Encephalopathy
    • None (1 point)
    • Grade 1-2 (2 points)
    • Grade 3-4 (3 points)
  • Interpretation
    • Class A (score 5-6)
      • Survival 100% (1 year); 85% (2 year)
    • Class B (score 7-9)
      • Survival 80% (1 year); 60% (2 year)
    • Class C (score 10-15)
      • Survival 45% (1 year); 35% (2 year)

  1. Schuppan D, Afdhal NH. Liver cirrhosis. Lancet. 2008;371(9615):838–851. Link to article
  2. Kockerling D, Nathwani R, Forlano R, Manousou P, Mullish BH, Dhar A. Current and future pharmacological therapies for managing cirrhosis and its complications. World J Gastroenterol2019; 25(8): 888-908. Link to article


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