Cirrhosis of the liver refers to scarring of the liver which results in abnormal liver function as a consequence of chronic (long-term) liver injury. Cirrhosis is a leading cause of illness and death in the United States. In the United States, approximately 5.5 million people (2% of the U.S. population) are affected by cirrhosis. Cirrhosis causes 26,000 deaths each year and is the seventh leading cause of death in the United States among adults between the ages of 25 and 64. It is expected that the number of people affected by cirrhosis will continue to increase in the near future.
The liver is the largest internal organ and is involved in many complex metabolic functions essential to life. Blood leaving the digestive system (stomach, intestines) passes through the liver on its way back to the heart. Essential functions of the liver include:
Cirrhosis of the liver is a consequence of long-term liver injury of many types. While excess alcohol use and chronic infection with hepatitis viruses (such as hepatitis B and hepatitis C) are the most common causes of cirrhosis in the United States, cirrhosis can be caused by many conditions including fatty liver disease, inherited disorders, drug-induced injury, bile duct disorders and autoimmune diseases. Some patients may have more than one cause for cirrhosis (such as alcohol excess and viral hepatitis). A large portion of patients (up to 20%) do not have an identifiable cause for cirrhosis. This is known as cryptogenic cirrhosis.
The signs and symptoms of liver cirrhosis may be absent or non-specific at early stages. Early non-specific symptoms include fatigue and itching. As scar tissue replaces healthy tissue and liver function worsens, a variety of liver-related symptoms may develop.
Since the liver performs many complex metabolic functions, there are many possible complications that can develop due to cirrhosis. In addition, some complications arise more commonly in certain diseases that cause cirrhosis (for example, osteoporosis occurs more commonly in patients with liver diseases that predominantly affect the bile ducts).
There are many causes of liver injury such as excessive alcohol consumption, viruses, inherited disorders, drug-related injury and environmental toxin exposure. Left untreated, any disease that causes on-going (chronic) liver injury can lead to cirrhosis. Injury to the liver leads to inflammation which may be detected by abnormalities in liver-related blood tests. Over time, ongoing injury leads to the development of scar tissue in the liver, a process called fibrosis. Since the liver has a substantial amount of reserve function, mild to moderate amounts of fibrosis usually do not lead to symptoms. However, as the amount of fibrosis increases it can lead to disruptions in the normal shape and function of the liver.
Cirrhosis occurs when the normal structure of the liver is disrupted by bands of scar tissue. One of the normal functions of the liver is to filter blood returning to the heart from the digestive system. When cirrhosis is present, the presence of scar tissue causes increased resistance to blood flow through the liver. This results in high pressures developing in the veins that drain into the liver, a process called portal hypertension. Many of the complications of liver disease, such as fluid retention and esophageal bleeding, are caused by the presence of portal hypertension.
There are several known risk factors for developing cirrhosis. The most common risk factors are:
Cirrhosis is best determined by examining a sample of liver tissue under the microscope, a procedure which is called a liver biopsy. In this relatively simple procedure a thin needle is inserted, usually under local anesthesia, into the liver and removes a small piece of liver tissue. Liver biopsy not only confirms the presence of cirrhosis, but can often provide information as to its cause.
A liver biopsy is not always necessary to diagnose cirrhosis. Frequently, your physician may be able to diagnose cirrhosis by the presence of changes noted during physical examination (such as enlargement of the spleen, enlargement of the breast tissue in men, and certain skin findings) together with the results from blood tests, imaging studies (such as ultrasound, CT or MRI scans) and/or endoscopy. There are several new tests that use ultrasound or MRI to directly measure the stiffness of the liver which may help in diagnosing cirrhosis but these tests are not widely available.
Medical care for patients with cirrhosis has several aims: (1) treating the underlying cause of liver disease when possible, (2) preventing cirrhosis-related complications, and (3) treating the symptoms of cirrhosis. Cirrhosis is a chronic disease, and patients with cirrhosis require ongoing medical care with a physician specializing in the care of patients with liver disease (a gastroenterologist or hepatologist).
Whenever possible, the underlying cause of cirrhosis should be treated. Some conditions improve with medial therapy and treatment can improve or delay worsening of liver function. In some circumstances, patients may not tolerate treatments for their underlying liver disease because their cirrhosis is too advanced.
Patients with cirrhosis should not drink alcohol. In patients who consume alcohol regularly, liver function may improve significantly with total avoidance of alcohol.
Your doctor may recommend various treatments aimed at preventing complications of cirrhosis from developing. Infection is an important cause of illness in patients with cirrhosis and your doctor may recommend updating your vaccinations. Typically, patients with cirrhosis should receive a yearly flu (influenza) vaccine and the pneumonia (pneumococcal) vaccine. Your doctor may test you for hepatitis A and hepatitis B and vaccinate you if you are not immune.
Patients with cirrhosis are at increased risk of developing primary liver cancer, known as hepatocellular carcinoma. The risk for liver cancer varies according to the underlying liver disease, but in patients with hepatitis C infection (one of the most common causes of liver disease in the United States) it is approximately 3-4% each year. Successful treatment for liver cancer depends on early detection. Liver cancers frequently do not cause any symptoms when they are small and treatment options may be limited by the time symptoms develop. Your doctor may recommend an ultrasound, CT scan or MRI of your liver at a regular interval, typically every 6 months, to detect liver tumors. A blood test called alpha-fetoprotein (AFP) may also be used to help detect tumors, however, it is not reliable enough by itself to replace the need for periodic imaging testing.
Your doctor may prescribe various treatments to help control symptoms from complications of cirrhosis. These may include:
In some cases your doctor may recommend the insertion of a TIPS shunt. The placement of a TIPS shunt is an invasive procedure. A TIPS shunt is a metal tube (also called a stent) placed within the liver under x-ray guidance through an incision in the jugular vein in the neck. A TIPS shunt works by decreasing the pressure against which blood must flow within the liver (that is to reduce portal hypertension). TIPS shunts are used to treat patients with severe difficulty with ascites or bleeding from varices that is not able to be controlled with medication or endoscopy. Not all patients should receive a TIPS shunt. TIPS shunt insertion has associated risks and the placement of TIPS shunt can lead to new or worsening hepatic encephalopathy.
For some patients with severe liver disease, liver transplantation may be considered as a treatment option. During liver transplantation surgery the diseased liver is removed and a new healthy liver from a deceased-donor or a part of a liver from a living-donor is put in its place. Liver transplantation surgery is a major undertaking and requires life-long anti-rejection medications afterwards. Extensive testing is required before a liver transplant to ensure that a candidate is in good enough health to proceed with a transplant operation. Additionally, transplant centers typically require some period of abstinence from alcohol (often at least 6 months) and/or formal alcohol and drug treatment for patients with alcohol-related liver disease before transplantation. Not all patients with cirrhosis need a liver transplant and transplantation is not the best choice for all patients. Although cirrhosis due to chronic hepatitis C is the leading cause for liver transplantation in the U.S., transplantation due to cirrhosis from fatty liver disease is increasing across the country. Because liver transplantation is so complex it is only performed at large specialty centers and your doctor may need to refer you elsewhere in order to be evaluated for a liver transplant.
William Sanchez, MD and Jayant A. Talwalkar, MD, MPH, FACG, Mayo College of Medicine, Rochester, MN – Published January 2009. Updated December 2012.