Cirrhosis

When injury to the liver is acute or liver damage is limited, the liver can usually repair itself. It is usually not short-term damage that causes cirrhosis, but repeated injury or damage occurring over many years that can lead to the development of cirrhosis. Causes are wide-ranging but generally fall into one of several categories:

• Alcoholic—excessive alcohol use over time can lead to alcoholic liver disease and cirrhosis.
• Associated with hepatitis, such as viral hepatitis, autoimmune hepatitis and non-alcoholic fatty liver disease (NAFLD)
• Biliary—obstruction and/or damage to bile ducts
• Cardiac—congestive heart failure over time can cause liver damage and cirrhosis
• Metabolic or inherited—these include diseases such as cystic fibrosis, haemochromatosis, Wilson disease, alpha-1 antitrypsin deficiency and Fanconi syndrome
• Drug-related (other than alcohol)
• Unknown—in about 10% of cases of cirrhosis, the cause is not known.

The frequency of these causes varies by population and geographic region. In the United States, about half of the cases of cirrhosis are caused by chronic hepatitis C infection and/or by chronic alcohol abuse (alcoholism). Chronic hepatitis B infection (sometimes with hepatitis D co-infection) causes a significant number of cases – and is one of the major causes in many parts of the world. Metabolic dysfunction-associated steatotic liver disease (MASLD) (previously called non-alcoholic fatty liver disease (NAFLD) and metabolic dysfunction-associated steatohepatitis (MASH) (previously called non-alcoholic steatohepatitis (NASH)) are significant non-infectious causes of cirrhosis, and the frequency of this cause is increasing while cases due to most other causes are remaining the same or decreasing.

Many of those who have cirrhosis have few to no symptoms. The liver compensates for some damage, and symptoms may not emerge until significant scarring is present. Symptoms may be nonspecific and include:

• Fatigue
• Weakness
• Confusion and difficulty concentrating
• Abdominal discomfort
• Itching
• Abdominal swelling (from fluid build-up, ascites)
• Jaundice
• Easy bleeding and bruising

 

It is important to detect cirrhosis as soon as possible since significant liver damage may occur with few or no symptoms. If the cause of liver damage can be eliminated or controlled, further scarring will stop and some existing scars may actually resolve. While blood tests can detect liver injury, there is no single test that can be used to diagnose cirrhosis. A liver biopsy is considered the "gold standard" for diagnosing cirrhosis, but the procedure is invasive and will not detect every case. Scans (such as CT, ultrasound or MRI) are less invasive and used to aide diagnosis.

Routine laboratory tests may be done to detect liver damage and/or scarring and to evaluate its severity, particularly if the individual has some risk factor for developing cirrhosis. Additional tests may be performed to help diagnose the underlying cause and to monitor the affected individual's health over time. This can include monitoring for the possible development of hepatocellular carcinoma.

Routine Tests

• Liver injury may be first detected in those who do not have symptoms during general health screening using a metabolic screen.
• Liver function tests (LFTs) may be performed when someone has symptoms that may be due to liver injury.

These panels include several liver tests: Other routine testing may include:

• Alanine aminotransferase (ALT) – an enzyme found mainly in the liver; may be moderately elevated with liver injury, including in cirrhosis.
• Aspartate aminotransferase (AST) – an enzyme found in the liver and other places; may be moderately elevated with liver injury, including in cirrhosis.
• Alkaline phosphatase (ALP) – an enzyme related to the bile ducts; often normal in people with cirrhosis
• Gamma-glutamyl transferase (GGT) – an enzyme found mainly in the liver; often normal in people with cirrhosis
• Total bilirubin – measures all the bilirubin in the blood; increased with many liver diseases but may be normal in cirrhosis until it is far advanced
• Albumin – a protein made by the liver, may be decreased in cirrhosis

If any of these tests are abnormal, then they will be further investigated. Typically, the pattern of results is more significant than the result from one or a few of these tests. A doctor may repeat them over a few days or weeks to determine if a pattern is present and to help gain clues to the underlying cause.

Other routine testing may include:

• Full blood count (FBC) – may be requested to evaluate a person's red and white blood cells and platelets; anaemia may be present if bleeding has occurred and the number of platelets is often decreased in cirrhosis.
• Prothrombin time (PT/INR) – most clotting factors are produced by the liver. This test evaluates clotting function and results may be prolonged with cirrhosis.

Many of the tests listed above may be used to monitor the progression of cirrhosis. As the condition worsens, results may become increasingly abnormal.

Follow-up Testing

• Hepatitis B and hepatitis C testing may be requested to help diagnose the underlying cause of chronic liver disease.
• If ascites is present, peritoneal fluid analysis may be performed.
• Liver biopsy involves taking a sample of liver tissue to evaluate the structure and cells of the liver. It can clearly indicate the presence of cirrhosis, but since the sample is tiny, a negative result may not rule cirrhosis out.

Depending on the suspected cause, one or more specialised tests may be performed:

• Iron tests – when haemochromatosis is suspected
• Copper and caeruloplasmin – when Wilson disease is suspected
• Antimitochondrial antibody (AMA) – to help diagnose PBC (primary biliary cirrhosis)
• Alpha-1 antitrypsin – to help diagnose a deficiency
• Genetic tests for inherited liver diseases (haemochromatosis, Wilson disease)
• Hyaluronic acid – associated with fibrosis; if low, then extensive fibrosis unlikely

Some tests may be requested to monitor for the development of complications:

• Alpha-fetoprotein (AFP) – often mildly elevated with cirrhosis; may be markedly elevated in liver cancer
• Des-gamma-carboxy prothrombin (DCP) – may be elevated in liver cancer
• Ammonia – occasionally requested – may be elevated in encephalopathy

Sometimes Requested

Calculations based upon panels of specific tests may be used to evaluate prognosis or likely cirrhosis:

• Child-Turcotte-Pugh (CTP) scoring system for cirrhosis - may be used to help evaluate life expectancy in those with advanced cirrhosis
• MELD (model of end-stage liver disease) - used to help determine those who are at a high risk of mortality, to consider for liver transplant
• Several commercially developed calculations (algorithms) are available to help recognise the presence and severity of scarring in the liver.

Non-Laboratory Tests

Other procedures and imaging tests may be useful:

• Ultrasound scan – sometimes requested to help diagnose metabolic dysfunction-associated steatotic liver disease (MASLD) (previously called non-alcoholic fatty liver disease (NAFLD)
• Once diagnosed with cirrhosis, periodic ultrasound scans to monitor for development of hepatocellular carcinoma (liver cancer)
• Magnetic or transient elastography – to evaluate degree of liver fibrosis by measuring liver stiffness

For more on these, visit Radiologyinfo.org.

Prevention

Steps can be taken to avoid and/or minimise the risk of certain forms of liver disease, thus preventing cirrhosis from developing. Some examples include preventing:

• Alcoholic liver disease – through alcohol moderation
• Hepatitis C infection through blood precautions, such as not sharing needles or personal items such as razors
• Hepatitis B infection through vaccination and taking precautions to avoid exposure
• Some cases of non-alcoholic fatty liver disease (NAFLD) by maintaining a healthy weight

Treatment

In individuals diagnosed with cirrhosis, treatment involves:

• Addressing and treating the underlying cause of the liver disease where possible
• Maintaining remaining liver function
• Treating complications

Those affected should avoid alcohol and should avoid substances that can harm the liver. They may need to modify or supplement their diet to ensure adequate nutrition and work with their doctor on medication doses as their liver may not be able to process drugs at a normal rate.

Endoscopy is sometimes needed to look for varices (dilated veins) and to address bleeding varices. In advanced cases of cirrhosis, a liver transplantation may be indicated.