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.
The liver and digestive system.
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 Minino 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. 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.
Reprinted from National Digestive Diseases Information Clearinghouse.
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