Cirrhosis of the Liver

Picture of liver tissue with cirrhosis.

Cirrhosis is a chronic (ongoing, long-term) disease of the liver. It means scarring to the normal liver tissue that keeps this important organ from working as it should. If the damage is not stopped, the liver gradually loses more of its ability to carry out its normal functions. This is called liver failure, sometimes referred to as end-stage liver disease.

The liver is the largest organ in the body and one of the most essential.

• It is about the size a football and is located on the right side in front, just below the lower rib cage.
• It produces substances that help fight infections and clot blood, filters toxins and infectious agents out of the blood, helps in the digestion of certain nutrients from foods, and stores energy for later use.
• These are just some of its many functions in the body.

The liver may be injured by a single event, as in acute (new, short-term) hepatitis; by regular injury over months or years, as in biliary tract blockage or chronic hepatitis; or by continuous injury, as in daily alcohol abuse.

• The liver responds to cell damage by producing strands of scar tissue that surround islands (nodules) of healing cells, making the liver knobby.
• At first, the inflammation in the liver causes it to swell. As the disease progresses and the amount of scar tissue in the liver increases, the liver will actually shrink.
• The scar tissue presses on the many blood vessels in the liver. This interrupts flow of blood to liver cells, which then die.
• Loss of liver cells hinders the liver's ability to perform its normal functions.

Loss of liver function affects the body in many ways. Cirrhosis, if severe enough, can cause many different complications. These can be severe, as follows:

Portal hypertension: The nodules and scar tissue can compress veins within the liver. This causes the blood pressure within the liver to be high, a condition known as portal hypertension.

• High pressures within blood vessels of the liver occur in a majority of people who have cirrhosis.
• Cirrhosis is the most common cause of portal hypertension in the United States.
• Portal hypertension may cause blood to back up in the intestines and other organs in the abdomen and cause bleeding into the intestines and fluid accumulation throughout the body.

Hepatic encephalopathy: In this condition, toxins build up in the bloodstream because the scarred liver is unable to rid them from the body.

• The toxins can cause you to behave bizarrely, to become confused, and to lose your ability to take care of yourself or others.
• Some people become very sleepy and cannot waken easily.

Gastrointestinal bleeding: Portal hypertension causes backing up of blood flow in the veins of the stomach and esophagus.

• This causes the veins to enlarge, forming "varices" (varicose veins).
• These varices can tear and bleed, and this bleeding can be life threatening.
• This usually shows up as vomiting bright red blood.

Infection: If you have cirrhosis, you are at risk for many infections because your liver cannot form the proteins needed to fight off infection.

Fluid retention (ascites): High pressures (portal hypertension) force fluid out of blood vessels in your liver, pooling it in your abdomen.

• Several liters of this fluid can pool in your abdomen, causing pain, swelling, difficulty breathing, and dehydration.
• As fluid pools in your abdomen, your kidneys will try to hold onto more water, because they think your body is dehydrated. The excess fluid collects in your lungs, legs, and abdomen.
• The fluid in your abdomen can become infected, called spontaneous bacterial peritonitis.

Hepatorenal syndrome: For unknown reasons, liver failure leads to kidney failure in some people.

• Often the progress toward liver failure is slow and gradual.
• Although cirrhosis has traditionally been linked with alcoholism, it has many causes. The most common causes in the United States are chronic alcoholism and hepatitis C.
• There is no cure for cirrhosis, but removing the cause can slow the disease. If the damage is not too severe, the liver can heal itself over time.

Jaundice - Yellowing of the skin and eyes from the deposition of bilirubin in these tissues. Bilirubin is a product of the breakdown of old blood cells in the liver.

Many people with cirrhosis have no symptoms during the early phases of the disease. Symptoms are caused by either of 2 problems:

• Gradual failure of the liver to carry out its natural functions
• Distortion of the liver's usual shape and size because of scarring

The most common symptoms of cirrhosis are as follows:

• Tiredness (fatigue) or even exhaustion
• Weakness
• Nausea
• Loss of appetite leading to weight loss
• Loss of sex drive

Symptoms may not appear until complications of cirrhosis set in. Many people do not know they have cirrhosis until they have a complication.

• Jaundice - Yellowing of the skin and eyes from the deposition of bilirubin in these tissues. Bilirubin is a product of the breakdown of old blood cells in the liver.
• Fever
• Vomiting
• Diarrhea
• Itching - From deposition in the skin of products of the breakdown of bile
• Abdominal pain - From an enlargement of the liver or formation of gallstones
• Abdominal swelling or bloating - From fluid retention
• Weight gain - From fluid retention
• Swelling in ankles and legs (edema) - From fluid retention
• Difficulty breathing - From fluid retention
• Sensitivity to medications - Due to impairment of the liver's ability to filter medications from the blood
• Confusion, delirium, personality changes, or hallucinations (encephalopathy) - From buildup of drugs or toxins in the blood, which then affect the brain
• Extreme sleepiness, difficulty awakening, or coma - Other symptoms of encephalopathy
• Bleeding from gums or nose - Due to impaired production of clotting factors
• Easy bruising - Due to impaired production of clotting factors
• Blood in vomit or feces - Due to bleeding of varicose veins caused by liver congestion
• Hemorrhoids - Varicose veins in the rectum due to liver congestion
• Loss of muscle mass (wasting)
• In women, abnormal menstrual periods - Due to impairment in hormone production and metabolism
• In men, enlargement of the breasts (gynecomastia), scrotal swelling, or small testes - Due to impairment in hormone production and metabolism

Cirrhosis can be caused by a number of conditions, including long-standing inflammation, poisons, infections, and heart disease, as well as chronic alcoholism and chronic hepatitis, the most common causes. For 30-50 percent of cirrhosis cases, however, no cause can be found. Often, the progress toward liver failure is slow and gradual. There is no cure for cirrhosis, but removing the cause can slow the sidease. If the damage is not too devere, the liver can heal itself over time.

Chronic alcoholism: Alcohol can poison all living cells, causing liver cells to become inflamed and die.

• The death of liver cells leads your body to form scar tissue around veins of your liver. Healing liver cells form nodules, which also press on the liver veins.
• This scarring process occurs in a significant percentage of alcoholics and is the most common form of cirrhosis in the United States.
• The severity of the process depends on how much you drink and how long you have been abusing alcohol. The amount of alcohol needed to injure the liver varies widely from individual to individual.
• Some families are more susceptible to cirrhosis than others.

Hepatitis: Hepatitis means inflammation of the liver from any cause, but it usually refers to a viral infection of the liver.

• Over many years the inflammation damages liver cells and leads to scarring.
• Hepatiti A, hepatitis B, hepatitis C, and hepatitis D all can cause cirrhosis.
• Worldwide, hepatitis B is the most common cause of cirrhosis, but in the United States hepatitis C is a more common cause.

Biliary cirrhosis: Bile is a substance produced by the liver to help the body digest fats.

• Bile is carried from the liver to the gallbladder and eventually into the intestines by small tubes called bile ducts.
• If these ducts become blocked, the bile backs up and can damage the liver. The liver becomes inflamed, starting the long process of cell damage that leads to cirrhosis.
• Children may be born with a condition that blocks the bile ducts called biliary atresia.
• This disease usually affects women aged 35-60 years.

Autoimmune cirrhosis: The body's immune system defends against "invaders" such as bacteria, viruses, or allergens.

• Autoimmune diseases occur when the immune system instead begins to fight healthy body tissues and organs.
• In autoimmune hepatitis, the body's immune system attacks the liver, causing cell damage that leads to cirrhosis.

Nonalcoholic fatty liver: This is a condition in which fat builds up in the liver, eventually causing scar tissue to form.

• This kind of cirrhosis is linked to diabetes, obesity, coronary artery disease, protein malnutrition, and treatment with corticosteroids such as prednisone.
• It is sometimes called "steatohepatitis."

Inherited diseases: A variety of genetic diseases can damage the liver.

• These are diseases that interfere with the metabolism of different substances by the liver.
• They include Wilson's disease, cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis, galactosemia, and glycogen storage disease.
• Most of these diseases are not common but they can be devastating.

Drugs, toxins, and infections: Various substances and germs can cause damage to the liver.

• Certain medications (for example, acetaminophen [Tylenol]), poisons, and environmental toxins can lead to cirrhosis.
• Reactions to certain drugs can damage the liver. This is rare.
• Long-term infections with various bacteria or parasites can damage the liver and cause cirrhosis.

Cardiac cirrhosis: Your heart is a pump that pushes blood throughout your body. When your heart doesn't pump well, blood "backs up" into the liver.

• This congestion causes damage to your liver.
• It may become swollen and painful. Later it becomes hard and less painful.
• The cause of the heart failure may be a heart valve problem, smoking, or infection of the heart muscle or the sac around the heart.

Your medical history, current symptoms, or physical exam findings may suggest to your health care provider that you have cirrhosis.

• He or she may suspect cirrhosis if you have abused alcohol or IV drugs in the past or still do so.
• Known chronic hepatitis, unexplained bleeding, jaundice, ascites (fluid building up in your abdomen), or any changes in the way you act are other findings that suggest cirrhosis.
• The condition may not be diagnosed until complications develop.

The steps in making the diagnosis of cirrhosis may include the following:

• Blood tests: To check whether the liver is functioning normally. Lab findings can be normal in cirrhosis, however.
• Ultrasound, CT scan: To look for signs of cirrhosis within or on the surface of the liver
• Liver biopsy: Removing tissue from the liver and studying it under a microscope to identify fibrosis and scarring. Biopsy is the only way diagnosis can be 100% certain.
• Laparoscope: A very tiny camera inserted through a small slit in the abdomen to view the liver directly. This may be done for another reason and your doctor finds you have cirrhosis.

If you have a major complication without knowing that you have cirrhosis, you will have to stay in the hospital. You will undergo tests and be treated for the complication.

If you have liver disease but no major complications, the work-up may be done on an outpatient basis if the following criteria are met:

• You have no signs or symptoms of infection.
• Your blood still has the ability to form clots and stop bleeding on its own.
• You are able to hold down foods and liquids.
• Your follow-up appointment with your health care provider is within 2 days.
• In the time between your diagnosis and follow-up visit, you will be in the company of an adult who can recognize complications and seek help should you become confused and unable to care for yourself.

Call your health care provider if you have symptoms that don't go away in a day or 2, or if you have any of these symptoms:

• Sudden weight gain with increased size of your abdomen
• Increasing water retention
• Jaundice
• Changes in your mental faculties or behavior
• New or different responses to medications
• Bleeding that takes longer than usual to stop

If you are unable to reach your health care provider or have any of these, go to the emergency department.

• Blood in your vomit or stool
• Difficulty breathing
• Abdominal pain
• Confusion or bizarre behavior
• Repeated vomiting
• Fever

Treatment for cirrhosis cannot reverse liver damage, but it can stop or slow progression of the disease causing it and reduce complications. Treatment depends on what is causing the cirrhosis and which particular complications, if any, have appeared.

• Stop drinking alcohol. If you stop all alcohol intake, you may slow the disease and feel better.
• Avoid medications that may be harmful to your liver, such as acetaminophen (Tylenol), or your kidneys, such as ibuprofen (Advil, etc). Ask you health care provider for a list.
• Cut down on salt if you are having problems with fluid retention. A low-sodium diet helps relieve that problem.
• Eat a balanced diet with adequate calories and protein. You also may want to take a daily multivitamin if your doctor agrees.
• If you ever have developed any brain disorder caused by your liver (hepatic encephalopathy), you should decrease your protein intake.

Most treatment for cirrhosis is directed toward relief of complications. Some underlying causes of cirrhosis, such as Wilson's disease, can be treated with medication.

• Many medicines have been studied, such as steroids, penicillamine (Cuprimine, Depen), and an anti-inflammatory agent (colchicine), but they have not been shown to prolong survival or improve survival rate.
• Researchers are studying various experimental treatments for cirrhosis.

Portal hypertension

Some people are treated with a drug called a beta-blocker to lower the pressure in the blood vessels.

Ascites

The slowing of blood flow through your liver increases the pressure in the blood vessels. This forces fluid out of the blood vessels and into other tissues, where it is retained.

• Your health care provider may prescribe water pills (a diuretic), which removes extra fluid from your body. This medication will make you urinate more often.
• Your health care provider may insert a needle into your abdomen to directly remove large amounts of fluid. However, the fluid usually collects again.
• If the fluid becomes infected, you will have to stay in the hospital and receive IV antibiotics.

Hepatic encephalopathy

If symptoms are severe, you will have to stay in the hospital, especially if you have become so confused you cannot care for yourself.

• You will be given lactulose, a drink that reduces the amounts of toxins absorbed into your intestinal tract.
• You may be started on a low-protein diet.
• Combining these 2 treatments improves symptoms in 75 percent of cases.

If symptoms are mild, you might be sent home and instructed to take lactulose every day and change to a low-protein diet, but to return if symptoms come back.

Clotting disorders

Adequate protein intake and vitamin supplements can help to correct clotting disorders.

Itching

Medications are available to reduce itching.

The only surgery that has been proven to improve the chances of long-term survival is liver transplantation.

• In this operation, the diseased liver is removed and replaced with a healthy liver from an organ donor.
• Most people who undergo liver transplantation survive.
• As in all transplantation procedures, supportive care before and after the procedure is very important in determining the success of the operation.

Portal hypertension

Various surgeries can be performed to redirect liver blood flow into the circulatory system, reducing liver blood pressures. However, surgery may worsen hepatic encephalopathy or ascites.

Bleeding varices

If you have bleeding from varices in the esophagus or stomach, you are at high risk of bleeding to death.

• You will have to stay in the hospital until the bleeding is under control.
• You have a 30% to 50% chance of dying during that hospital stay if you suffer from bleeding varicose veins in your esophagus. About 80% of patients will have more than one bleeding episode.

If you have significant blood loss, treatment will focus on restoring lost fluids.

• You will be monitored carefully until bleeding is controlled and your blood circulation is stabilized.
• Two large IV lines will be placed to replace lost fluids.
• You will need supplemental oxygen until you begin to replace some of the lost blood.
• You may need blood transfusions.

Ongoing bleeding from the esophagus is detected by inserting a tube down your nose into your stomach to suck out any pooled blood. Once bleeding is recognized, various methods are used to control it.

• Balloon inflation to compress the vein
• Medications that decrease blood flow into the liver
• Tying off the bleeding vein

Hepatorenal syndrome

For unknown reasons, liver failure sometimes leads to kidney failure.

• Kidney failure often causes other organs throughout the body to fail. This can be fatal.
• Liver transplant is the only treatment that works in this advanced disease.

Liver cancer

People with ongoing liver damage often develop liver cancer before they develop cirrhosis.

• People with liver cancer may die within 3-6 months after diagnosis if the cancer remains untreated.
• Even with treatment, people rarely survive beyond 5 years.
• Surgery is the only chance for a cure, but if detected late, the cancer may have progressed too far by the time surgery is performed.
• Liver transplantation may also be considered.

The best way to avoid cirrhosis is to avoid the underlying conditions that cause it.

• Know the risk factors for hepatitis B and hepatitis C and avoid them as much as possible.
• Avoid risky behaviors such as alcohol abuse, IV drug use, and unprotected sexual intercourse.
• Drink alcohol only in moderation, if at all.
• Develop healthy habits. Avoid using tobacco. Eat a healthy diet, get plenty of physical activity and rest, and maintain your weight in a healthy range.
• Talk to your health care provider before taking vitamin supplements. Large doses of vitamins and minerals, especially vitamin A, iron, or copper, can actually worsen liver damage.
• Hepatitis B immunizations are available to health care workers and others at high risk of contacting the disease. Immunization of all American children against hepatitis B, now required, will reduce the incidence of cirrhosis in the future.
• No effective hepatitis C vaccination is available.

Your recovery depends on the cause of your cirrhosis and whether you are able to remove or stop the cause. Liver transplantation remains the best treatment, but livers are available for a limited number of people.

American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038
(800) 465-4837

Hepatitis Foundation International
504 Blick Drive
Silver Spring, MD 20904-2901
(800) 891-0707 or (301) 622-4200

United Network for Organ Sharing (UNOS)
1100 Boulders Parkway, Suite 500
P.O. Box 13770
Richmond, VA 23225-8770
(800) 24-DONOR or (804) 330-8500

American Gastroenterological Association

American Liver Foundation

Hepatitis Foundation International

National Digestive Diseases Information Clearinghouse, Cirrhosis of the Liver 

United Network for Organ Sharing