Cirrhosis refers to severe, permanent scarring of the liver that results from certain liver diseases.1 Healthy liver tissue becomes scar tissue, eventually preventing the liver from performing its necessary functions.
The National Institutes of Health (NIH) estimates that 1 in 400 adults in the United States have cirrhosis.1 This number is likely higher, as many people living with cirrhosis are undiagnosed. Cirrhosis is more common in adults between 45 and 54 years of age. About 117 million people live with compensated cirrhosis (without symptoms) worldwide as of 2017.2
Another article will cover the special considerations for cirrhosis in children.
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Symptoms, Signs, and Stages of Cirrhosis
Symptoms and signs of cirrhosis depend on the stage of the disease. Generally, liver disease is divided into four stages4-5:
- Hepatitis (liver inflammation)
- Fibrosis (scarring): Scarring or stiffening of the liver after longstanding hepatitis; due to high levels of collagen (connective tissue protein) buildup.
- Cirrhosis (severe scarring): Permanent damage to the liver caused by severe scarring.
- Liver Failure or Liver Cancer: Liver failure means the liver is no longer functioning and a liver transplant is needed. Liver cancer is another end-stage form of liver disease.
Cirrhosis usually has no symptoms when liver disease first reaches this stage.6 If someone has early symptoms, these can include:
- Feeling tired or weak
- Low appetite
- Unintentional weight loss
- Nausea and vomiting
- Mild upper right belly pain
Once cirrhosis is severe and affects liver function, more serious symptoms can develop6-7:
- Bleeding or bruising easily
- Confusion, difficulty thinking, memory loss
- Sleep changes
- Personality changes
- Leg edema (swelling of legs, ankles, or feet)
- Ascites (fluid buildup in the belly)
- Severely itchy skin
- Dark urine
- Jaundice (yellowing of eyes or skin)
- Gastrointestinal hemorrhage (bleeding in the stomach or gut)
Cirrhosis that causes symptoms is called decompensated cirrhosis.7
Causes of Cirrhosis and Risk Factors
The most common causes of cirrhosis are also the most common risk factors for developing it:
Alcoholic Liver Disease
Heavy alcohol use is defined by the Centers for Disease Control and Prevention as more than 8 drinks per week for women and 15 or more drinks per week for men8. Heavy alcohol use significantly increases the risk of developing alcoholic liver disease. Alcoholic liver disease begins with steatosis (fat buildup in the liver) and eventually becomes fibrosis and cirrhosis.
Nonalcoholic Fatty Liver Disease (NAFLD)
Nonalcoholic fatty liver disease (NAFLD) includes nonalcoholic fatty liver (NAFL, fat buildup in the liver above 5 percent) and nonalcoholic steatohepatitis (NASH, liver cell inflammation due to fat buildup).9 Chronic NASH can progress to fibrosis and cirrhosis.
Chronic Hepatitis B
Hepatitis B is a virus that mainly infects the liver.10 This infection most commonly spreads through body fluid exposure such as unprotected sex or sharing needles with someone who has it. The virus is also passed from mother to baby. When hepatitis B becomes chronic, the inflammation can lead to fibrosis and cirrhosis. This change is more likely if someone is diagnosed with hepatitis B in childhood.
Chronic Hepatitis C (HCV)
Hepatitis C is another virus that targets the liver.11 It becomes chronic in 75 to 85 percent of people who have an acute (occurring over a short period) hepatitis C infection. Chronic hepatitis C can cause fibrosis and cirrhosis of the liver. Like hepatitis B, it’s usually spread through body fluid exposure through unprotected sex or sharing needles with an infected person. Hepatitis C is also passed from mother to baby.
Other Causes of Cirrhosis
The following are less common causes of cirrhosis:7
- Autoimmune hepatitis is a form of hepatitis that results from antibodies attacking liver cells.18 Antibodies are proteins made by white blood cells to attack foreign substances. In autoimmune conditions, these antibodies attack body cells.
- Primary biliary cholangitis (PBC) is an autoimmune liver disease where antibodies destroy intrahepatic bile ducts (tubes within the liver that transport bile), leading to decreased liver function.19
- Primary sclerosing cholangitis (PSC) is a chronic liver disease where inflammation and fibrosis destroy bile ducts inside and outside the liver.20 The underlying cause is unknown.
- Hemochromatosis is a liver disorder that develops when the body absorbs too much iron (a substance used by the body to make red blood cells and carry oxygen).21
- Alpha-1 antitrypsin (AAT) deficiency is a genetic disorder where the AAT protein does not work.22 Normally, AAT protects the lungs from the immune system. In AAT deficiency, lung cells are damaged, and the defective AAT builds up in the liver and causes damage.
Testing and Diagnosis of Cirrhosis
Diagnosing cirrhosis requires a thorough history and exam by a doctor, along with testing.12 A doctor will ask questions to identify symptoms of cirrhosis and then perform an exam to find any signs of disease. They will also ask about other health conditions that increase the risk of developing cirrhosis.
Blood Tests for Cirrhosis
Since cirrhosis has no symptoms in the early stages, testing is important to diagnose cirrhosis early. If cirrhosis is suspected, a doctor will order certain blood tests13:
Liver tests: Measure levels of certain enzymes (substances that speed up chemical reactions in the body). If these enzyme levels are outside the normal range, it may be a sign that the liver is damaged. Liver tests with the following results suggest cirrhosis:
- Increased levels of aspartate transaminase (AST) and alanine transaminase (ALT) enzymes
- Increased bilirubin (a protein released by the liver in bile that forms when red blood cells are broken down)
- Decreased levels of specific proteins that are made by the liver
Complete blood count (CBC): A blood test that measures red and white blood cells and platelets. This test can show signs of infection or blood loss due to complications of cirrhosis.
Viral tests: Tests that measure amounts of viruses in the blood can identify a cause for cirrhosis.
Autoimmune tests: Tests that measure proteins, including antibodies (proteins made by the immune system to attack foreign substances), that are indicators of autoimmune liver diseases can identify a cause for cirrhosis.
Fibrosis blood tests: Tests that combine multiple labs to provide a general idea of the level of fibrosis in the liver.
Imaging Studies for Cirrhosis
Along with blood tests, certain imaging studies are important for diagnosing cirrhosis7:
Liver biopsy: The gold standard diagnostic test for cirrhosis. A biopsy is used to give the disease a grade (amount of inflammation) and a stage (amount of fibrosis). To diagnose cirrhosis with a biopsy, nodules and fibrosis must be present.
Ultrasound (US): A useful first imaging test to look for cirrhosis. Ultrasound works by sending sound waves through the body to image organs. US shows liver nodularity (scarring in the liver) and increased liver size. These are nonspecific findings (shared with other liver diseases) but can show the first signs of cirrhosis.
Doppler Ultrasound: A type of ultrasound that looks at blood vessels. It’s used in cirrhosis to determine how well blood flows through the veins of the liver.
Computed tomography (CT) and magnetic resonance imaging (MRI): Used to find hepatocellular carcinoma (HCC or liver cancer) and blood vessel problems. MRI is better at finding these, but it is expensive and not always available.
Transient elastography (TE, or fibroscan): A high-speed US used to help diagnose cirrhosis by measuring liver stiffness related to fibrosis.
Complications of Cirrhosis
Because the liver performs many important functions, cirrhosis causes significant problems throughout the body.7 The major complications of cirrhosis are14:
Ascites: Fluid buildup in the belly; about 85% of people with it have cirrhosis.
Spontaneous bacterial peritonitis (SBP): People with ascites are at risk of developing SBP, a bacterial infection of the ascitic fluid.
Variceal bleeding: Increased pressure in the liver’s blood vessels can lead to varices (blood vessel swellings) in the esophagus and stomach. These swellings can cause serious internal bleeding.
Hepatorenal syndrome: Refers to kidney disease due to cirrhosis. The cause of hepatorenal syndrome is not completely understood, but it is likely due to low blood flow to the kidneys.
Hepatic encephalopathy: A change in mental status due to the buildup of ammonia (a substance made when proteins break down) in cirrhosis. When liver function suffers, it’s unable to process ammonia correctly. This releases ammonia in the blood. Ammonia causes confusion, personality changes, and, in severe cases, coma.
Portal hypertension: High blood pressure in the blood vessels of the liver. Liver scarring narrows blood vessels and causes high pressure. Portal hypertension leads to ascites, SBP, internal bleeding, and hepatorenal syndrome.
Treatment Options and Prevention of Cirrhosis
Options for treating cirrhosis depend on the stage of the disease. At early stages, treatments can improve or reverse fibrosis.15 Even at late stages, there are medications that treat cirrhosis and its complications.
Treatment of compensated cirrhosis focuses on the underlying cause of the disease.15 Recommendations for treating the most common causes of cirrhosis are reviewed here.
Chronic hepatitis C is treated with 12 to 24 weeks of a direct-acting antiviral drug (DAA). This treatment cures most chronic hepatitis C.16
Chronic hepatitis B is also treated with antiviral medication. According to the World Health Organization (WHO), treatment can slow cirrhosis’s development and reduce the risk of getting HCC.17 The WHO suggests that doctors use either tenofovir or entecavir for lifelong treatment.
Alcoholic liver disease is treated with abstinence from alcohol.23 Doctors refer people to alcohol treatment programs when they have trouble quitting. Importantly, people who drink alcohol regularly should talk with their doctor before quitting due to the risk of withdrawal seizures.24
NAFLD and NASH are treated with a balanced diet (including vegetables, fruits, low-fat protein, whole grains, and healthy fats) and physical activity.23 The goal of treatment is to reduce the amount of fat in the liver.
Autoimmune hepatitis is treated with corticosteroids (medications that reduce inflammation and block the immune system) and other immune system-blocking drugs.18 The treatment of choice has historically been prednisone. Other drug combinations have replaced prednisone due to its side effects.
PBC is treated with ursodiol, a medication that protects liver cells from bile damage.19 People benefit most when this treatment is started early. Another medication called obeticholic acid is used in patients with a poor response to, or who can’t take, ursodiol.
There is no FDA-recommended treatment for PSC.20 To relieve symptoms, a procedure can be done to enlarge the common bile duct (the large tube into which smaller ducts drain). People can also undergo surgery where the damaged ducts are bypassed. Liver transplantation is the only known cure for PSC.
Hemochromatosis is treated with phlebotomy (blood draws) once or twice per week.21 Phlebotomy helps reduce iron toxicity by removing red blood cells, the main way that iron moves through the body.
High-risk AAT deficiency (risk determined by genetic testing) is treated with weekly AAT replacement.22
Treatment of decompensated cirrhosis involves both the treatment of underlying disease and complication management.14
To treat portal hypertension, a doctor may prescribe medications to lower blood pressure.25 If varices (enlarged veins in the esophagus and/or stomach) develop and begin bleeding, an upper endoscopy (a procedure that uses a camera to look at the esophagus) can stop the bleeding.
When fluid builds up in the arms, legs, or belly, medications can be used to remove fluid.25 Salt intake should also be limited. For ascites—a buildup of fluid in the abdomen—a doctor can use a needle to remove fluid and test it for infection. If spontaneous bacterial peritonitis (SBP), a type of fluid infection, is diagnosed, the treatment is antibiotics.
In people who develop hepatic encephalopathy, medications are given to reduce the amount of ammonia in the blood and improve symptoms.25
Once the disease progresses to liver failure, a liver transplant is the only curative treatment option.25
The tools to prevent cirrhosis aim to prevent liver disease or the worsening of diagnosed liver disease. Strategies to prevent liver disease and its progression include:
- Having a balanced diet
- Exercising regularly
- Treating other diseases that increase the risk of developing liver disease, like diabetes and viral hepatitis
- Avoiding alcohol
- Having protected sex
- Getting vaccinated against hepatitis B
- Regularly visiting the doctor for screenings and early diagnosis of liver disease
Resources for Living with Cirrhosis
If you or someone you love has cirrhosis, you probably have a lot of questions and concerns. Thankfully, there are many resources available online to help manage this disease. Look at these resources to get started: