Cirrhosis is a condition in which the liver slowly deteriorates and
malfunctions due to chronic injury. Scar tissue replaces healthy liver tissue,
partially blocking the flow of blood through the liver. Scarring also impairs
the liver's ability to

 
• control infections
• remove bacteria and toxins from the blood
• process nutrients, hormones, and drugs
• make proteins that regulate blood clotting
• produce bile to help absorb fats—including cholesterol—and fat-soluble
  vitamins

A healthy liver is able to regenerate most of its own cells when they become
damaged. With end-stage cirrhosis, the liver can no longer effectively replace
damaged cells. A healthy liver is necessary for survival.

Cirrhosis is the twelfth leading cause of death by disease, accounting
for 27,000 deaths each year.1 The condition
affects men slightly more often than women.

1 Miniño AM, Heron MP, Murphy SL, Kochanek KD. Deaths: Final data for 2004.
Centers for Disease Control and Prevention Web site.
http://www.cdc.gov/nchs/data/nvsr/nvsr55/nvsr55_19.pdf
(PDF, 3450 KB). Updated October 10, 2007. Accessed January 20, 2008.


 What causes cirrhosis?
Cirrhosis has various causes. In the United States, heavy alcohol consumption
and chronic hepatitis C have been the most common causes of cirrhosis. Obesity
is becoming a common cause of cirrhosis, either as the sole cause or in
combination with alcohol, hepatitis C, or both. Many people with cirrhosis have
more than one cause of liver damage.

Cirrhosis is not caused by trauma to the liver or other acute, or short-term,
causes of damage. Usually years of chronic injury are required to cause
cirrhosis.

Alcohol-related liver disease.
Most people who consume alcohol do not suffer damage to the liver. But heavy
alcohol use over several years can cause chronic injury to the liver. The amount of
alcohol it takes to damage the liver varies greatly from person to person. For women,
consuming two to three drinks—including beer and wine—per day and for men, three
to four drinks per day, can lead to liver damage and cirrhosis. In the past,
alcohol-related cirrhosis led to more deaths than cirrhosis due to any other
cause. Deaths caused by obesity-related cirrhosis are increasing.

Chronic hepatitis C.
The hepatitis C virus is a liver infection that is spread by contact with an infected person's blood.
Chronic hepatitis C causes inflammation and damage to the liver over time that can lead
to cirrhosis.

Chronic hepatitis B and D.
The hepatitis B virus is a liver infection that is spread by contact with an infected person's blood,
semen, or  other body fluid. Hepatitis B, like hepatitis C, causes liver inflammation and
injury that can lead to cirrhosis. The hepatitis B vaccine is given to all
infants and many adults to prevent the virus. Hepatitis D is another virus that
infects the liver and can lead to cirrhosis, but it occurs only in people who
already have hepatitis B.

Nonalcoholic fatty liver disease (NAFLD).
In NAFLD, fat builds up in the liver and eventually causes cirrhosis. This increasingly common
liver disease is associated with obesity, diabetes, protein malnutrition, coronary artery disease,
and corticosteroid medications.

Autoimmune hepatitis.
This form of hepatitis is caused by the body's immune system attacking liver cells and
causing inflammation, damage, and eventually cirrhosis. Researchers believe genetic
factors may make some people more prone to autoimmune diseases. About 70 percent of those with
autoimmune hepatitis are female.

Diseases that damage or destroy bile ducts.
Several different diseases can damage or destroy the ducts that carry bile from the
liver, causing bile to back up in the liver and leading to cirrhosis. In adults,
the most common condition in this category is primary biliary cirrhosis, a
disease in which the bile ducts become inflamed and damaged and, ultimately,
disappear. Secondary biliary cirrhosis can happen if the ducts are mistakenly
tied off or injured during gallbladder surgery. Primary sclerosing cholangitis
is another condition that causes damage and scarring of bile ducts. In infants,
damaged bile ducts are commonly caused by Alagille syndrome or biliary atresia,
conditions in which the ducts are absent or injured.

Inherited diseases.
Cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis, Wilson disease,
galactosemia, and glycogen storage diseases are inherited diseases that interfere
with how the liver produces, processes, and stores enzymes, proteins, metals, and
other substances the body needs to function properly. Cirrhosis can result from these conditions.

Drugs, toxins, and infections.
Other causes of cirrhosis include drug reactions, prolonged exposure to toxic chemicals, parasitic
infections, and repeated bouts of heart failure with liver congestion.

What are the symptoms of cirrhosis?
Many people with cirrhosis have no symptoms in the early stages of the
disease. However, as the disease progresses, a person may experience the
following symptoms:

 
• weakness
• fatigue
• loss of appetite
• nausea
• vomiting
• weight loss
• abdominal pain and bloating when fluid accumulates in the abdomen
• itching
• spiderlike blood vessels on the skin

What are the complications of cirrhosis?
As liver function deteriorates, one or more complications may develop. In
some people, complications may be the first signs of the disease.

Edema and ascites. When liver damage progresses to an
advanced stage, fluid collects in the legs, called edema, and in the abdomen,
called ascites. Ascites can lead to bacterial peritonitis, a serious
infection.

Bruising and bleeding. When the liver slows or stops
producing the proteins needed for blood clotting, a person will bruise or bleed
easily.

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, which increases the pressure in the portal vein. This
condition is called portal hypertension.

Esophageal varices and gastropathy. When portal hypertension
occurs, it may cause enlarged blood vessels in the esophagus, called varices, or
in the stomach, called gastropathy, or both. Enlarged blood vessels are more
likely to burst due to thin walls and increased pressure. If they burst, serious
bleeding can occur in the esophagus or upper stomach, requiring immediate
medical attention.

Splenomegaly. When portal hypertension occurs, the spleen
frequently enlarges and holds white blood cells and platelets, reducing the
numbers of these cells in the blood. A low platelet count may be the first
evidence that a person has developed cirrhosis.

Jaundice. Jaundice occurs when the diseased liver does not
remove enough bilirubin from the blood, causing yellowing of the skin and whites
of the eyes and darkening of the urine. Bilirubin is the pigment that gives bile
its reddish-yellow color.

Gallstones. If cirrhosis prevents bile from flowing freely
to and from the gallbladder, the bile hardens as gallstones.

Sensitivity to medications. Cirrhosis slows the liver's
ability to filter medications from the blood. When this occurs, medications act
longer than expected and build up in the body. This causes a person to be more
sensitive to medications and their side effects.

Hepatic encephalopathy. A failing liver cannot remove toxins
from the blood, and they eventually accumulate in the brain. The buildup of
toxins in the brain—called hepatic encephalopathy—can decrease mental function
and cause coma. Signs of decreased mental function include confusion,
personality changes, memory loss, trouble concentrating, and a change in sleep
habits.

Insulin resistance and type 2 diabetes. Cirrhosis causes
resistance to insulin—a hormone produced by the pancreas that enables the body
to use glucose as energy. With insulin resistance, the body's muscle, fat, and
liver cells do not use insulin properly. The pancreas tries to keep up with the
demand for insulin by producing more, but excess glucose builds up in the
bloodstream causing type 2 diabetes.

Liver cancer. Hepatocellular carcinoma is a type of liver
cancer that can occur in people with cirrhosis. Hepatocellular carcinoma has a
high mortality rate, but several treatment options are available.

Other problems. Cirrhosis can cause immune system
dysfunction, leading to the risk of infection. Cirrhosis can also cause kidney
and lung failure, known as hepatorenal and hepatopulmonary syndromes.

How is cirrhosis diagnosed?
The diagnosis of cirrhosis is usually based on the presence of a risk factor
for cirrhosis, such as alcohol use or obesity, and is confirmed by physical
examination, blood tests, and imaging. The doctor will ask about the person's
medical history and symptoms and perform a thorough physical examination to
observe for clinical signs of the disease. For example, on abdominal
examination, the liver may feel hard or enlarged with signs of ascites. The
doctor will order blood tests that may be helpful in evaluating the liver and
increasing the suspicion of cirrhosis.

To view the liver for signs of enlargement, reduced blood flow, or ascites,
the doctor may order a computerized tomography (CT) scan, ultrasound, magnetic
resonance imaging (MRI), or liver scan. The doctor may look at the liver
directly by inserting a laparoscope into the abdomen. A laparoscope is an
instrument with a camera that relays pictures to a computer screen.

A liver biopsy can confirm the diagnosis of cirrhosis but is not always
necessary. A biopsy is usually done if the result might have an impact on
treatment. The biopsy is performed with a needle inserted between the ribs or
into a vein in the neck. Precautions are taken to minimize discomfort. A tiny
sample of liver tissue is examined with a microscope for scarring or other signs
of cirrhosis. Sometimes a cause of liver damage other than cirrhosis is found
during biopsy. 

How is the severity of cirrhosis measured?
The model for end-stage liver disease (MELD) score measures the severity of
cirrhosis. The MELD score was developed to predict the 90-day survival of people
with advanced cirrhosis. The MELD score is based on three blood tests:

• international normalized ratio (INR)—tests the clotting tendency of
  blood
• bilirubin—tests the amount of bile pigment in the blood
• creatinine—tests kidney function

MELD scores usually range between 6 and 40, with a score of 6 indicating the
best likelihood of 90-day survival. 

How is cirrhosis treated?
Treatment for cirrhosis depends on the cause of the disease and whether
complications are present. The goals of treatment are to slow the progression of
scar tissue in the liver and prevent or treat the complications of the disease.
Hospitalization may be necessary for cirrhosis with complications.

Eating a nutritious diet. Because malnutrition is common in
people with cirrhosis, a healthy diet is important in all stages of the disease.
Health care providers recommend a meal plan that is well balanced. If ascites
develops, a sodium-restricted diet is recommended. A person with cirrhosis
should not eat raw shellfish, which can contain a bacterium that causes serious
infection. To improve nutrition, the doctor may add a liquid supplement taken by
mouth or through a nasogastric tube—a tiny tube inserted through the nose and
throat that reaches into the stomach.

Avoiding alcohol and other substances. People with cirrhosis
are encouraged not to consume any alcohol or illicit substances, as both will
cause more liver damage. Because many vitamins and medications—prescription and
over-the-counter—can affect liver function, a doctor should be consulted before
taking them.

Treatment for cirrhosis also addresses specific complications. For edema and
ascites, the doctor will recommend diuretics—medications that remove fluid from
the body. Large amounts of ascitic fluid may be removed from the abdomen and
checked for bacterial peritonitis. Oral antibiotics may be prescribed to prevent
infection. Severe infection with ascites will require intravenous (IV)
antibiotics.

The doctor may prescribe a beta-blocker or nitrate for portal hypertension.
Beta-blockers can lower the pressure in the varices and reduce the risk of
bleeding. Gastrointestinal bleeding requires an immediate upper endoscopy to
look for esophageal varices. The doctor may perform a band-ligation using a
special device to compress the varices and stop the bleeding. People who have
had varices in the past may need to take medicine to prevent future
episodes.

Hepatic encephalopathy is treated by cleansing the bowel with lactulose—a
laxative given orally or in enemas. Antibiotics are added to the treatment if
necessary. Patients may be asked to reduce dietary protein intake. Hepatic
encephalopathy may improve as other complications of cirrhosis are
controlled.

Some people with cirrhosis who develop hepatorenal failure must undergo
regular hemodialysis treatment, which uses a machine to clean wastes from the
blood. Medications are also given to improve blood flow through the kidneys.

Other treatments address the specific causes of cirrhosis. Treatment for
cirrhosis caused by hepatitis depends on the specific type of hepatitis. For
example, interferon and other antiviral drugs are prescribed for viral
hepatitis, and autoimmune hepatitis requires corticosteroids and other drugs
that suppress the immune system.

Medications are given to treat various symptoms of cirrhosis, such as itching
and abdominal pain. 

When is a liver transplant indicated for cirrhosis?
A liver transplant is considered when complications cannot be controlled by
treatment. Liver transplantation is a major operation in which the diseased
liver is removed and replaced with a healthy one from an organ donor. A team of
health professionals determines the risks and benefits of the procedure for each
patient. Survival rates have improved over the past several years because of
drugs that suppress the immune system and keep it from attacking and damaging
the new liver.

The number of people who need a liver transplant far exceeds the number of
available organs. A person needing a transplant must go through a complicated
evaluation process before being added to a long transplant waiting list.
Generally, organs are given to people with the best chance of living the longest
after a transplant. Survival after a transplant requires intensive follow-up and
cooperation on the part of the patient and caregiver.
 

Points to Remember

 
• Cirrhosis is a condition in which the liver slowly deteriorates and
  malfunctions due to chronic injury. Scar tissue replaces normal, healthy liver
  tissue, preventing the liver from working as it should.
• In the United States, heavy alcohol consumption and chronic hepatitis C have
  been the most common causes of cirrhosis. Obesity is becoming a common cause of
  cirrhosis, either as the sole cause or in combination with alcohol, hepatitis C,
  or both. Many people with cirrhosis have more than one cause of liver
  damage.
• Other causes of cirrhosis include hepatitis B, hepatitis D, and autoimmune
  hepatitis; diseases that damage or destroy bile ducts, inherited diseases, and
  nonalcoholic fatty liver disease; and drugs, toxins, and infections.
• Many people with cirrhosis have no symptoms in the early stages of the
  disease. As the disease progresses, symptoms may include weakness, fatigue, loss
  of appetite, nausea, vomiting, weight loss, abdominal pain and bloating,
  itching, and spiderlike blood vessels on the skin.
• As liver function deteriorates, one or more complications may develop. In
  some people, complications may be the first signs of the disease.
• The goals of treatment are to stop the progression of scar tissue in the
  liver and prevent or treat complications.
• Treatment for cirrhosis includes avoidance of alcohol and other drugs,
  nutrition therapy, and other therapies that treat specific complications or
  causes of the disease.
• Hospitalization may be necessary for cirrhosis with complications.
• A liver transplant is considered when complications of cirrhosis cannot be
  controlled by treatment.

 Hope through Research
The National Institute of Diabetes and Digestive and Kidney Diseases'
Division of Digestive Diseases and Nutrition supports basic and clinical
research into liver diseases—including cirrhosis—and liver transplantation.
Researchers are also studying

 
• the mechanisms of cirrhosis reversal in the early stages of the disease
• potential new approaches to the management of complications of
  cirrhosis
• the long-term outcome of new drugs to treat portal hypertension
• the development of therapies to prevent and treat the recurrence of
  hepatitis C after liver transplantation

Participants in clinical trials can play a more active role in their own
health care, gain access to new research treatments before they are widely
available, and help others by contributing to medical research. For information
about current studies, visit www.ClinicalTrials.gov.
 
For More Information
American Liver Foundation
75 Maiden Lane, Suite  603
New York, NY 10038–4810
Phone: 1–800–GO–LIVER (465–4837) or
212–668–1000
Fax: 212–483–8179
Email: info@liverfoundation.org
Internet: www.liverfoundation.org 

Hepatitis Foundation International
504 Blick Drive
Silver Spring, MD 20904–2901
Phone: 1–800–891–0707 or
301–622–4200
Fax: 301–622–4702
Email: hfi@comcast.net
Internet: www.hepfi.org 

United Network for Organ Sharing
  P.O. Box 2484  
Richmond, VA 23218
  Phone: 1–888–894–6361 or 804–782–4800
  Fax: 804–782–4817

  Internet: www.unos.org 

You may also find additional information about this topic by visiting
MedlinePlus at www.medlineplus.gov.

This publication may contain information about medications. When prepared,
this publication included the most current information available. For updates or
for questions about any medications, contact the U.S. Food and Drug  
Administration toll-free at 1–888–INFO–FDA (1–888–463–6332) or visit www.fda.gov.
Consult your doctor for more  information.


  Acknowledgments
Publications produced by the Clearinghouse are carefully reviewed by both
NIDDK scientists and outside experts. This publication was reviewed by Bruce A.
Runyon, M.D., Loma Linda University Medical Center.

http://digestive.niddk.nih.gov/ddiseases/pubs/cirrhosis/