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.
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.
