Fulminant Hepatitis

Aka: Fulminant Hepatitis, Acute Hepatic Failure, Acute Liver Failure

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II. Definitions

  1. Fulminant Hepatitis
    1. Rapid onset and progression within weeks to liver necrosis with secondary Hepatic Encephalopathy and Coagulopathy
  2. Acute Liver Failure
    1. Course of liver disease <=26 weeks, with INR >1.5, Hepatic Encephalopathy and no prior evidence of liver disease

III. Epidemiology

  1. Incidence: 2000 cases per year in the United States

IV. Pathophysiology

  1. Massive hepatic necrosis over the course of days to weeks
  2. Results in rapid progression from Jaundice to encephalopathy and Coagulopathy
  3. Multiorgan failure including Acute Renal Failure follows

V. Types

  1. Timing based on pregression from Jaundice onset to encephalopathy
  2. Acute Liver Failure within 1 week
    1. Hyperacute liver failure
  3. Acute Liver Failure within 1-4 weeks
    1. Acute Liver Failure
  4. Acute Liver Failure over >5-8 weeks
    1. Subacute liver failure

VI. Causes

  1. See Acute Hepatitis
  2. See Hepatotoxin
  3. Infectious Disease
    1. Viral Hepatitis
      1. Hepatitis A
      2. Hepatitis B
      3. Hepatitis C
      4. Hepatitis D
    2. Bacterial Infection
    3. Rickettsial infection
    4. Parasitic Infection
  4. Toxic Hepatitis
    1. Hepatotoxin exposure or other drug-induced cause
    2. Examples: Acetaminophen Overdose, Amanita muscaria ingestion
  5. Acute Ischemic Liver Injury (shock liver)
  6. Budd-Chiari Syndrome
  7. Idiopathic Chronic Active Hepatitis
  8. Wilson's Disease (Acute)
  9. Microvesicular Steatosis (Fat) Syndromes
    1. Nonalcoholic Fatty Liver
    2. Acute Fatty Liver of Pregnancy
    3. Reye's Syndrome

VII. Symptoms

  1. Vomiting
  2. Upper Abdominal Pain
  3. Anorexia
  4. Jaundice

VIII. Signs

  1. Neurologic changes (Hepatic Encephalopathy)
    1. Altered Level of Consciousness (Delirium, coma)
    2. Decerebrate rigidity (with severe cerebral edema)
    3. Personality change
  2. Jaundice
  3. Coagulopathy
    1. Bleeding (e.g. Gastrointestinal Bleeding)
  4. Acute Renal Failure (Hepatorenal Syndrome)
  5. Hypoglycemia
  6. Acute Pancreatitis
  7. Cardiopulmonary failure
  8. Ascites (due to Portal Hypertension)

IX. Labs

  1. See Acute Hepatitis

X. Imaging

  1. See Acute Hepatitis

XI. Management

  1. Targeted therapy
    1. Delivery for pregnancy related Acute Liver Disease (especially Acute Fatty Liver of Pregnancy)
    2. Withdraw all known Hepatotoxins
    3. Treat known Hepatotoxin exposures
      1. Consult with poison control and hepatology
      2. Consider empiric N-Acetylcysteine in possible acute Toxic Hepatitis
        1. Effective beyond Acetaminophen Overdose or Amanita muscaria ingestion
        2. Duration typically longer (>24 hours) than for Acetaminophen Overdose (per poison control)
  2. Supportive care
    1. ABC Management
      1. Endotracheal Intubation often required
    2. Fluid and Electrolytes
      1. Volume expansion with crystalloid initially, but avoid Fluid Overload
        1. Consider Albumin 25% at 50-100 ml aliquot or Albumin 5% at 250 ml aliquot
        2. Liver failure is associated with hypoalbuminemia
      2. Correct acid-base status and Electrolyte abnormalities
      3. Monitor Serum Glucose
        1. Correct Hypoglycemia with IV D10 or D20 prn
      4. Hemorrhagic Shock
        1. Consider FFP or PCC4 for Coagulopathy and severe active bleeding
        2. INR is not an accurate measure of bleeding risk in the absence of Warfarin
      5. Vasopressors
        1. Consider Vasopressin as a first-line Vasopressor in liver failure
    3. Prevent GI Bleed
      1. H2 Blockers to maintain gastric pH >3.5
    4. Monitor for infection
      1. Complicated by Bacterial or fungal infection in 80% of cases
      2. Infection is often occult with non-specific changes in status (e.g. worsening encephalopathy)
      3. Routinely monitor urine, Chest XRay and other markers of infection
      4. Have low threshold to start Antibiotics and Antifungals
      5. Consider prophylactic Antibiotics (e.g. Ceftriaxone)
    5. Hepatic Encephalopathy
      1. Increased risk for cerebral edema, intracranial Hypertension and Uncal Herniation
      2. Monitor Hepatic Encephalopathy patients in ICU
      3. Hepatic Encephalopathy may be more severe in Acute Liver Failure than in longstanding Cirrhosis
      4. Obtain Head CT and Ocular Ultrasound for Optic Nerve Sheath Diameter
      5. General measures
        1. Consider Endotracheal Intubation
        2. Elevate head of bed to 30 degrees
        3. Control systemic Hypertension
        4. Lactulose (oral, rectal) lowers cerebral ammonia and may decrease ICH
      6. Other measures for lowering Intracranial Pressure (questionable efficacy unless temporizing for procedure)
        1. Lower Intracranial Pressure with Mannitol IV or Hypertonic Saline prn (while replacing urine losses)
  3. Transfer to center capable of performing Liver Transplant (if potential candidate)
    1. See Liver Transplant Center Referral Indications
    2. Other Liver Transplant referral indications
      1. Grade 3-4 Encephalopathy
      2. Adverse prognostic indicators as above

XII. Prognosis: Factors associated with poor outcomes

  1. Advanced age
  2. Halothane exposure
  3. Hepatitis C
  4. Coma (80% Mortality)
  5. Rapid decrease in liver span
  6. Respiratory Failure
  7. Marked ProTime prolongation
  8. Factor V Level <20%

XIII. References

  1. Swaminathan and Weingart in Herbert (2020) EM:Rap 20(10):1-2
  2. Swencki (2015) Crit Dec Emerg Med 29(11):2-10
  3. Swencki (2023) Crit Dec Emerg Med 37(8):4-12

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