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Liver cirrhosis
Other Resources UpToDate PubMed

Liver cirrhosis

Contributors: Diane Kuhn MD, Michael W. Winter MD, Paritosh Prasad MD, Sara Manning MD
Other Resources UpToDate PubMed

Synopsis

Emergent Care / Stabilization:
Cirrhosis is a chronic condition involving fibrosis of the liver. While uncomplicated cirrhosis does not require emergency management, individuals with cirrhosis are at risk for decompensated liver disease, which may be characterized by ascites, hepatic encephalopathy, and/or variceal bleeding. Patients with decompensated cirrhosis presenting to the emergency department may be hypotensive, have altered mental status, and be at higher risk for infection. Researchers have distinguished between decompensated cirrhosis and acute or chronic liver failure, with the latter characterized by extrahepatic organ failure.

After performing a history and physical examination, the emergency physician should assess volume status and resuscitate the patient accordingly. Mean arterial pressure goals may be slightly slower than in patients without liver disease, typically 60 mm Hg compared to 65 mm Hg. Fluid resuscitation should be approached cautiously, and administration of albumin may be appropriate. If fluid resuscitation does not restore adequate perfusion, norepinephrine is the vasopressor of choice. Administration of hydrocortisone should also be considered given high rates of adrenal insufficiency in this patient population. Hypotensive patients with decompensated liver disease should be treated as potentially septic, with administration of broad-spectrum antibiotics. Delay of antibiotics is associated with increased mortality. In addition to typical sepsis workup and management, physicians should pay careful attention to abdominal tenderness and/or presence of a fluid wave that could suggest spontaneous bacterial peritonitis. In the case of a variceal bleed, patients may require airway protection.

Diagnosis Overview:
Cirrhosis is thought to be a mostly irreversible process, which can lead to acute decompensated cirrhosis with or without acute or chronic liver failure. Decompensated cirrhosis is hallmarked by ascites development with risk for spontaneous bacterial peritonitis, hepatic encephalopathy, variceal formation and bleeding, hepatorenal syndrome, and hepatocellular carcinoma. Extrahepatic organ failure is characteristic of acute or chronic liver disease, which is thought to be related to increased systemic inflammation and oxidative stress. Some examples of extrahepatic failure include:
  • Hepatic encephalopathy – A reversible set of neuropsychiatric symptoms secondary to neurotoxins such as ammonia.
  • Hepatorenal syndrome – Concurrent renal failure and liver disease despite histologically normal kidneys; studies implicate the renin-angiotensin-aldosterone system and resulting vasoconstriction.
  • Hepatopulmonary syndrome – Dilation of pulmonary vasculature secondary to portal hypertension and/or advanced liver disease, resulting in reduced arterial O2 saturation.
  • Cirrhotic cardiomyopathy – Cardiac dysfunction, particularly under stress, in patients with advanced liver disease and no known cardiac disease.
All of these decompensating events contribute to a high mortality from cirrhosis and a greatly diminished life expectancy for patients who do not receive a liver transplant. Cirrhosis is thought to be mostly irreversible.

Patients with cirrhosis also have an increased risk of infectious complications including urinary tract infection, pneumonia, spontaneous bacteremia, skin and soft tissue infections (eg, Staphylococcus aureus), candidiasis, Clostridioides difficile infection, cholangitis, and tuberculosis. Of patients with decompensated liver disease, 10%-13% have fungal infections, and suspicion for these is increased in the setting of negative bacterial cultures.

The most common etiologies of cirrhosis are chronic viral hepatitis B and hepatitis C, nonalcoholic steatohepatitis, and alcoholic liver disease.

Cirrhosis is a progressive condition that greatly decreases life expectancy. Due to its irreversibility, patients either receive a liver transplant or are at high risk of developing a decompensating event, which carries high mortality.

Cirrhosis is much less common in pediatric patients than adults and, when present, is typically secondary to a congenital disease such as biliary atresia. Presentation may differ, with generalized symptoms such as failure to thrive, weakness, and decreased appetite.

Codes

ICD10CM:
K74.60 – Unspecified cirrhosis of liver

SNOMEDCT:
19943007 – Cirrhosis of liver

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Etiologies of cirrhosis:
  • Chronic viral hepatitis (Hepatitis A virus infection, Hepatitis B virus infection, or Hepatitis C virus infection)
  • Autoimmune hepatitis
  • Alcoholic hepatitis
  • Metabolic dysfunction-associated steatotic liver disease
  • Hemochromatosis
  • Alpha-1 antitrypsin deficiency
  • Wilson disease
  • Hepatocellular carcinoma
  • Celiac disease
  • Hypothyroidism or Hyperthyroidism
  • Primary biliary cholangitis
  • Primary sclerosing cholangitis
  • Biliary obstruction
  • Budd-Chiari syndrome
  • Cystic fibrosis
  • Glycogen storage diseases (Glycogen storage disease type 1, Glycogen storage disease type 2, Glycogen storage disease type 3, Glycogen storage disease type 4, Glycogen storage disease type 5, Glycogen storage disease type 6, Glycogen storage disease type 7)
  • Liver fluke (Fascioliasis, Clonorchiasis, Opisthorchis viverrini infection)
  • Echinococcosis
  • Schistosomiasis
  • Congestive heart failure (cardiohepatic syndrome)
  • Malignancy (particularly metastasis to liver and Liver cancer, Gastric cancer, and Cholangiocarcinoma)
  • Sarcoidosis
  • Amyloidosis (AL amyloidosis, AA amyloidosis)
  • Polycystic liver disease
  • Tuberculosis
Differential diagnosis for emergent presentations of liver disease:
  • Altered mental status – The differential diagnosis for altered mental status in the emergency department includes infectious etiologies (Bacterial meningitis, Bacterial sepsis), vascular (Cerebellar stroke), metabolic or electrolyte changes (Hyponatremia, Diabetic ketoacidosis, Hyperosmolar hyperglycemic state), and primary neurologic conditions such as Frontotemporal lobar degeneration, among others.
  • GI bleeding (GI) – While GI bleeding may be related to varices, it can also be caused by many other conditions including Peptic ulcer disease, Diverticulitis, Hemorrhoids, Angiodysplasia of the colon, malignancies, and Anal fissure.
  • Hypotension – Causes of hypotension or shock can include obstructive shock (Pulmonary embolism), Septic shock (sepsis), Cardiogenic shock, neurogenic shock, or hypovolemic shock (dehydration, traumatic hemorrhage).
  • Volume overload – In addition to liver disease, Chronic kidney disease and cardiac conditions such as Congestive heart failure can lead to volume retention with lower extremity edema, pleural effusions, and symptoms such as dyspnea and orthopnea.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:11/18/2023
Last Updated:12/07/2023
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Liver cirrhosis
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Copyright © 2024 VisualDx®. All rights reserved.