Cirrhosis of the Liver

Alternative names: Chronic liver disease, alcoholic liver disease

Cirrhosis of the liver is a condition in which scar tissue replaces normal, healthy tissue, blocking the flow of blood through the organ and preventing it from working as it should.

Cirrhosis is the eighth leading cause of death by disease and the cost of cirrhosis in terms of human suffering, hospital costs and lost productivity is high because loss of liver function affects the body in many ways.

Causes and Development

In the United States, chronic alcoholism and hepatitis C are the most common of its many causes.

Alcoholic liver disease (ALD) is a condition in which there is acute or chronic inflammation of the liver caused by excessive intake of alcohol.  Damage to the liver progresses through three stages:

1. fatty liver disease (liver becomes yellow and enlarges), which is reversible once alcohol intake has stopped
2. alcoholic hepatitis (widespread inflammation and death of liver cells), which is sometimes reversible
3. cirrhosis (dead liver cells are replaced by scar tissue), which is irreversible

A healthy liver (left) compared to a cirrhotic liver (right) which exhibits scar tissue and regenerative nodules

Cirrhosis is the end stage of chronic liver disease in which much of the functional liver tissue has been replaced by fibrous tissue, and eventually leads to liver failure.  Often there are no symptoms of cirrhosis until the disease is far advanced.

Autoimmune hepatitis is caused by a problem with the immune system.

Inherited diseases including Alpha-1 antitrypsin deficiency, hemochromatosis, Wilson's disease, galactosemia, and glycogen storage diseases interfere with the way the liver produces, processes, and stores enzymes, proteins, metals, and other substances the body needs to function properly.

In cases of Nonalcoholic steatohepatitis (NASH) or Non-alcoholic fatty liver disease (NAFLD), fat builds up in the liver and eventually causes scar tissue.  This type of hepatitis appears to be associated with diabetes, protein malnutrition, excess sugar intake, poor diet in general, obesity, coronary artery disease, and corticosteroid treatment.

Blocked bile ducts. When the ducts that carry bile out of the liver are blocked, bile backs up and damages liver tissue.  In babies, blocked bile ducts are most commonly caused by biliary atresia, a disease in which the bile ducts are absent or injured.  In adults, the most common cause is primary biliary cirrhosis, a disease in which the ducts become inflamed, blocked, and scarred.  Secondary biliary cirrhosis can happen after gallbladder surgery, if the ducts are inadvertently tied off or injured.

Drugs, toxins, and infections. Severe reactions to prescription drugs, prolonged exposure to environmental toxins, the parasitic infection schistosomiasis, and repeated bouts of heart failure with liver congestion can each lead to cirrhosis.

Signs and Symptoms

Many people with cirrhosis have no symptoms in the early stages of the disease.  However, as scar tissue replaces healthy cells, liver function starts to fail and a person may experience various symptoms.  As the disease progresses, complications may develop.

Symptoms include:

• Ascites
• Swelling of the legs
• Vomiting blood
• Confusion
• Jaundice
• Small, red spider-like blood vessels on the skin
• Loss of appetite
• Weakness
• Weight loss
• Nausea and vomiting
• Impotence and loss of interest in sex
• Bleeding hemorrhoids
• Exhaustion
• Fatigue

Additional symptoms that may be associated with this disease:

• Urine output, decreased
• Swelling, overall
• Stools, pale or clay colored
• Nosebleed or bleeding gums
• Gynecomastia (breast development in males)
• Abdominal pain
• Abdominal indigestion
• Fevers

Symptoms may develop gradually, or there may be no symptoms.

Diagnosis and Tests

The doctor may diagnose cirrhosis on the basis of symptoms, laboratory tests, the patient's medical history and a physical examination.  The doctor may, for example, notice that the liver feels harder or larger than usual and order blood tests that can show whether liver disease is present.

A physical examination may reveal an enlarged liver or spleen, distended abdomen, yellow eyes or skin (jaundice), red spider-like blood vessels on the skin, excess breast tissue, small testicles in men, contracted fingers, or dilated abdominal wall veins.

A liver biopsy involves injecting local anesthetic, a small incision, and extracting a liver tissue sample using a hollow needle

If a liver examination is necessary, the doctor might order a computerized axial tomography (CAT) scan, ultrasound, a scan of the liver using a radioisotope, or look at the liver using a laparoscope.  A liver biopsy will confirm the diagnosis.  For a biopsy, the doctor uses a needle to take a small sample of tissue from the liver, then examines it for scarring or other signs of disease.

Tests can reveal liver problems including:

• Anemia (detected on a CBC)
• Coagulation abnormalities
• Elevated liver enzymes
• Elevated bilirubin
• Serum albumin low
• Enlarged liver (seen with an abdominal X-ray.)

This disease may also alter the results of the following tests:

• Cholesterol test
• Complement
• Alpha-fetoprotein
• Renin
• Serum magnesium

Treatment and Prevention

For those diagnosed with liver cirrhosis, it is extremely important to stop drinking alcohol, even if alcohol is not the cause

Liver damage from cirrhosis cannot be reversed, but treatment can stop or delay further progression and reduce complications.  Treatment should always include complete alcohol avoidance, but also depends on the cause of cirrhosis and any complications a person is experiencing.  For example, hepatitis-related cirrhosis is treated with the different types of hepatitis medication, such as interferon for viral hepatitis and corticosteroids for autoimmune hepatitis; cirrhosis caused by Wilson's disease, in which copper builds up in organs, is treated with medications to remove the copper.  Treatment will also include remedies for complications such as edema, infections and itching.

Treatment is directed at managing the complications of cirrhosis and preventing further liver damage.

• Offending medications and alcohol are stopped.
• Bleeding varices are treated by upper endoscopy with banding or sclerosis.
• Ascites (excess abdominal fluid) is treated with diuretics, fluid and salt restriction, and removal of fluid (paracentesis).
• Coagulopathy may be treated with blood products or vitamin K.
• Encephalopathy is treated with the medication lactulose – sometimes antibiotics are used and patients should avoid a diet high in protein.
• Infections are treated with antibiotics.
• If cirrhosis progresses and becomes life-threatening, a liver transplant should be considered.

The stress of illness can often be eased by joining a support group whose members share common experiences and problems.

Prevention
Don't drink heavily.  If you find that your drinking is getting out of hand, seek professional help.  Avoiding intravenous drug use (or only using clean needles and never sharing other equipment) will reduce the risk of hepatitis B and C. Some research indicates that hepatitis C may be spread via shared use of straws or items used to snort cocaine or other drugs.  Avoid snorting drugs or sharing any related paraphernalia.  If you have a problem with illicit drugs, seek help.

Prognosis; Complications; Seek medical attention if...

Survival depends on the severity of complications of cirrhosis and the underlying causes.

When complications cannot be controlled or when the liver becomes so damaged from scarring that it completely stops functioning, a liver transplant is necessary.

Other types of complication:

Edema and ascites. When the liver loses its ability to make the protein albumin, water accumulates in the leg (edema) and abdomen (ascites).

Bruising and bleeding. When the liver slows or stops production of the proteins needed for blood clotting, a person will bruise or bleed easily.  For example, bleeding esophageal varices.

Jaundice. Jaundice is a yellowing of the skin and eyes that occurs when the diseased liver does not absorb enough bilirubin.

Itching. Bile products deposited in the skin may cause intense itching.

Gallstones. If cirrhosis prevents bile from reaching the gallbladder, a person may develop gallstones.

Toxins in the blood or brain. A damaged liver cannot remove toxins from the blood, causing them to accumulate in the blood (sepsis) and eventually the brain.  There, toxins can dull mental functioning and cause personality changes, coma, and even death.  Signs of the buildup of toxins in the brain include neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, mental confusion, or changes in sleep habits.

Sensitivity to medication. Cirrhosis slows the liver's ability to filter medications from the blood.  Because the liver does not remove drugs from the blood at the usual rate, they act longer than expected and build up in the body.  This causes a person to be more sensitive to medications and their side-effects.  Hence the "Tell your doctor if you have liver problems..."  statement on drug ads.

Portal hypertension. Normally, blood from the intestines and spleen is carried to the liver through the portal vein.  But cirrhosis slows the normal flow of blood through the portal vein, which increases the pressure inside it.  This condition is called portal hypertension.

Varices. When blood flow through the portal vein slows, blood from the intestines and spleen backs up into blood vessels in the stomach and esophagus.  These blood vessels may become enlarged because they are not meant to carry this much blood.  The enlarged blood vessels, called varices, have thin walls and carry high pressure, and thus are more likely to burst.  If they do burst, the result is a serious bleeding problem in the upper stomach or esophagus that requires immediate medical attention.

Problems in other organs. Cirrhosis can cause immune system dysfunction, leading to infection.  Ascites (fluid retention) in the abdomen may become infected with bacteria normally present in the intestines (bacterial peritonitis), and cirrhosis can also lead to kidney dysfunction and failure.

Other possible complications of cirrhosis include:

• Hepatic encephalopathy
• Liver cancer (hepatocellular carcinoma)
• Kidney failure (hepatorenal syndrome)

Call your health care provider if symptoms develop that are suggestive of cirrhosis.  Go to the emergency room if vomiting blood or if rapid changes in alertness or consciousness occur.