Liver Cirrhosis

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Medical Author: Jeffrey A Gunter, MD. Coauthor(s): Erik D Barton, MD, MS
Medical Editors: Scott H Plantz, MD, FAAEM; Francisco Talavera, PharmD, PhD; Steven L Bernstein, MD
Cirrhosis Overview

Cirrhosis is a chronic (ongoing, long-term) disease of the liver. It means damage 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 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 absorption of certain nutrients from foods, and stores energy for later use.
    
  • These are just some of its many functions in the body.
    
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• 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.
    
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• 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 60% of people who have cirrhosis.
    
  • Cirrhosis is the most common cause of portal hypertension in the United States.
    
  • Portal hypertension may cause bleeding into the intestines and fluid accumulation throughout the body.
    
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• Hepatic encephalopathy: In this condition, toxins build up in the bloodstream becausethe 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.
    
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• 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 blood.
    
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• 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.
    
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• Hepatorenal syndrome: For
  unknown reasons, liver failure leads to kidney failure in some people
  

• Often the progress toward liver failure is slow and gradual.
  
• After heart disease and cancer,
  cirrhosis is the third most common cause of death in people aged 45-65
  years in the United States. It is a leading cause of death by disease
  overall. An estimated 25,000 people die of cirrhosis in the United
  States each year.
  
• Although cirrhosis has traditionally been linked with alcoholism, it has many causes. The most common causes in the United States are chronic alcoholism andhepatitis 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.
  
• Cirrhosis Causes
  
  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.
  
• 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 10-20 percent 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.
    
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• 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.
    
  • 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.
    
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• 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.
    
  • In adults, gallstones are a common cause of bile duct blockage. Surgery to remove the gallbladder also blocks the bile ducts.
    
  • Children may be born with a condition that blocks the bile ducts called biliary atresia.
    
  • This disease usually affects women aged 35-60 years.
    
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• 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.
    
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• 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."
    
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• 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.
    
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• 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.
    
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• 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.
    
  • Cirrhosis Symptoms
    
  • 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
      
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  </li>
  
• 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
    
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• 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 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 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 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 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
    
  • When to Seek Medical Care
    
    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.
  
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• Blood in your vomit or stool
  
• Difficulty breathing
  
• Abdominal pain
  
• Confusion or bizarre behavior
  
• Repeated vomiting
  
• Fever
  
• Exams and Tests
  
  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 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, or radioisotope scan - To look for signs of cirrhosis within or on the surface of the liver
  
• Laparoscope - A very tiny camera inserted through a small slit in the abdomen to view the liver directly
  
• 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.
  
• Cirrhosis Treatment
  
  Treatment for cirrhosis cannot reverse liver damage, but it can stop
  or slow progression of the disease and reduce complications. Treatment
  depends on what is causing the cirrhosis and which particular
  complications, if any, have appeared
  

Self-Care at Home

• 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.
  
• Medical Treatment
  
  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
.Surgery
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.
  
• About 80-90 percent of 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 1 in 2 chance of dying during that hospital stay if you suffer from bleeding varicose veins in your esophagus.
  

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 usually the cancer has progressed too far by the time surgery is performed.
  
• Liver transplantation may also be considered.
  
• Next Steps
  Follow-up
  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.
  
• Prevention
  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.
  
• Outlook
  
  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 very few people.Only
  50 percent of people with severe alcoholic cirrhosis survive 2 years,
  and only 35 percent survive 5 years. Quitting drinking alcohol will slow
  the progress of the disease and may prevent complications. Your chance
  of recovery worsens after the onset of complications