Cirrhosis

Description
An in-depth report on the causes, diagnosis, treatment, and prevention of cirrhosis.
Highlights
Causes of Cirrhosis
Cirrhosis is a liver disease characterized by permanent scarring of the liver that
interferes with its normal functions. Causes include:
Alcoholism
Chronic hepatitis B and C
Autoimmune hepatitis
Bile duct disorders such as primary biliary cirrhosis and primary sclerosing
cholangitis
Nonalcoholic fatty liver disease (NAFLD), which includes nonalcoholic
steatohepatitis (NASH)
Metabolic disorders such as hemachromatosis, Wilsons disease, and alpha-1
antitrypsin deficiency
Prolonged exposure to certain chemicals and medications
Complications
Cirrhosis can cause many serious complications including:
Ascites (fluid buildup in the abdomen)
Variceal hemorrhage, severe bleeding from varices (enlarged veins in the
esophagus and upper stomach)
Spontaneous bacterial peritonitis, a severe infection of the membrane lining of
the abdomen
Hepatic encephalopathy, impaired mental function caused by buildup in the body
of toxins such as ammonia
Hepatocellular carcinoma, a type of liver cancer
Hepatorenal syndrome, when kidney failure occurs along with severe cirrhosis
Dietary and Lifestyle Changes
All patients with cirrhosis can benefit from certain lifestyle interventions. These include:
Stop drinking alcohol.
Restrict dietary salt.
Follow a good diet plan, which may include protein restriction.
Get vaccinations for influenza, hepatitis A and B, and pneumococcal pneumonia
(if recommended by your doctor).
Inform your doctor of all prescription and nonprescription medications, and any
herbs and supplements, you take or are considering taking.
Treatment
Cirrhosis is an irreversible condition. Treatment focuses on slowing the progression of
liver damage and reducing the risk of further complications. Your doctor will treat any
underlying medical conditions that are the cause of your cirrhosis. If liver damage
progresses to liver failure, patients may be candidates for liver transplantation. Liver
donations can come from either a cadaver or from a living donor. Patients with cirrhosis
who have a liver transplant have very good chances for survival.

Introduction
Cirrhosis is an irreversible result of various disorders that damage liver cells over time.
Eventually, damage becomes so extensive that the normal structure of the liver is
distorted and its function is impaired.

Cirrhosis is a chronic liver disease that is the result of damage to liver tissue with
scarring of the liver (fibrosis - nodular regeneration) causing progressive decrease in
liver function, excessive fluid in the abdomen (ascites), bleeding disorders
(coagulopathy), increased pressure in the blood vessels (portal hypertension), and brain
function disorders (hepatic encephalopathy). Excessive alcohol use is the leading cause
of cirrhosis.
Scarring. The main damage in cirrhosis is triggered by scarring (fibrosis) that occurs
from injuries due to alcohol, viruses, or other assaults. The scar tissue and other
changes in liver cells gradually replace healthy liver tissue and act like small dams to
alter the flow of blood and bile in and out of the liver.
Altered Blood and Bile Flow. The changes in blood and bile flow have significant
consequences, with both the liver and other organs responding to the altered flow:
The small blood vessels and bile ducts in the liver itself narrow (constrict). Blood
vessels in other organs, including the kidney, also narrow.
Blood flow coming from the intestine into the liver is slowed by the narrow blood
vessels. It backs up through the portal vein and seeks other routes.
Enlarged, abnormally twisted and swollen veins called varices form in the
stomach and lower part of the esophagus to transport the blood diverted from the liver.
Bilirubin also builds up in the bloodstream, resulting in jaundice, a yellowish cast
in the skin and eyes, as well as dark-colored urine.
Fluid buildup in the abdomen (called ascites), and swelling in the legs
(edema) are common.
Functions of the Liver

The liver is the largest internal organ in the body. In the healthy adult, it weighs about 3
pounds. The liver is wedge-shaped, with the top part wider than the bottom. It is located
right below the diaphragm and occupies the entire upper right quadrant of the abdomen.
The liver performs over 500 vital functions. Damage to the liver can impair these and
many other processes. Among them are the following:
Processing Healthful Nutrients. The liver processes all of the nutrients the body
requires, including proteins, glucose, vitamins, and fats.
Producing Proteins The liver is the bodys factory where many important proteins are
made. The blood protein albumin is one example that is often underproduced in patients
with cirrhosis.
Producing Bile. The liver produces bile, a green-colored fluid that helps the body absorb
fats and fat-soluble vitamins. Bile contains bilirubin, a yellow-green pigment produced
from the breakdown of hemoglobin, the oxygen-carrying component in red blood cells.
Bile also contains bile salts, fatty acids, cholesterol, and other substances.
Bile travels from the liver to the gallbladder, where it is stored until after a meal. It is then
secreted into the intestines where it helps digest fat. Because bile can also travel
directly from the liver to the intestines, patients who have had their gallbladders
removed can still absorb fat normally.
Eliminating Toxins. One of the liver's major functions is to render harmless potentially
toxic substances, including alcohol, ammonia, drugs, and harmful by-products of
digestion.
Structure of the Liver
The vital processes the liver performs rely on well-organized liver architecture.
The basic building blocks of the liver are the following structures:
Bile ducts
Blood vessels
Working liver tissue (called the parenchyma)
Supportive (connective) tissue
The liver is a built on a framework of lobes:
Lobes. The liver is divided into two major lobes, a right and a smaller left, which
are separated by tough, fibrous connective tissue.
Lobules. The liver's two major lobes contain about 100,000 smaller lobes, called
lobules. Each lobule contains microscopic columns of liver cells and blood vessels.
Bracing the corners of each lobule column are an artery and a vein that carry blood and
a bile duct that drains bile.
Bile ducts. The bile ducts in the column corners collect bile draining from tiny
canals around the liver cells. These ducts eventually join to form the large common bile
duct that leads from the liver to the gallbladder.
Arteries and veins. The arteries bring oxygen-rich blood to nourish the liver cells.
The veins supply the liver cells with blood containing the nutrients and toxins that the
liver cells process. A central vein runs through each column and collects the processed
blood from both sources. These veins join to form the hepatic vein.
The Liver's Blood Supply. The liver is rich in blood. Its vessels contain about a pint, or
13% of the body's supply. It gets its blood from two large vessels, the hepatic

artery and the portal vein, and is drained of blood by the hepatic vein. (The word
"hepatic" derives from the Latin word for liver.)
The hepatic artery. This artery carries blood from the heart directly to the liver. This
blood nourishes the liver.
The portal vein. The portal vein carries blood that has circulated through the stomach,
spleen, and intestine to the liver. The liver processes this blood, extracting nutrients and
toxins.
The hepatic vein. This vein carries blood away from the liver and connects to
the inferior vena cava, a large vein that carries blood back to the heart.

Click the icon to see an image of the liver.

Causes
Several processes can lead to cirrhosis.
Alcoholism
Chronic alcoholism particularly endangers the liver by causing alcoholic liver disease
(also called alcohol-induced liver disease). Alcoholic liver disease includes fatty liver
(build-up of fat cells in the liver), alcoholic hepatitis (inflammation of the liver caused by
heavy drinking), and alcoholic cirrhosis. Alcoholic cirrhosis is the primary type of
cirrhosis in the U.S. It develops in 10 - 20% of heavy drinkers, usually after 10 - 15
years of heavy alcohol consumption. People who drink heavily and who also have
hepatitis C are at particular risk of developing cirrhosis. In the liver, alcohol converts to
toxic chemicals that trigger inflammation and tissue injury, which lead to cirrhosis.
Chronic Viral Hepatitis
Chronic viral hepatitis, both hepatitis B and hepatitis C, is another primary cause of
cirrhosis. Chronic hepatitis C is a more common cause of cirrhosis in developed
countries, while hepatitis B is a more common cause of cirrhosis worldwide, especially
in sub-Saharan Africa and parts of Asia. People with chronic hepatitis B who are coinfected with hepatitis D are especially at risk for cirrhosis. The longer a patient has had
chronic hepatitis, the greater the risk for eventually developing cirrhosis.
Hepatitis viruses can produce inflammation in liver cells, causing injury or destruction. If
the condition is severe enough, the cell damage becomes progressive, leading to scar
tissue in the liver. In advanced cases, the liver shrivels in size, a condition called
postnecrotic or posthepatic cirrhosis.

Hepatitis C is a virus-caused liver inflammation which may lead to jaundice, fever, and
cirrhosis. The people most at risk for contracting and spreading hepatitis C are those
who share needles for injecting drugs and health care workers or emergency workers
who may be exposed to contaminated blood.
Autoimmune Hepatitis
Autoimmune hepatitis, like other autoimmune disorders, develops when a misdirected
immune system attacks the body's own cells and organs. People who have autoimmune
hepatitis also often have other autoimmune conditions, including systemic lupus
erythematosus, rheumatoid arthritis, Sjgren syndrome, scleroderma, inflammatory
bowel disease, glomerulonephritis, and hemolytic anemia. Autoimmune hepatitis
typically occurs in women ages 15 - 40.
Bile Ducts Disorders
Disorders that block or damage the bile ducts can cause bile to back up in the liver,
leading to inflammation and cirrhosis. These diseases include primary biliary cirrhosis
and primary sclerosing chlorangitis.
Primary Biliary Cirrhosis. Up to 95% of primary biliary cirrhosis (PBC) cases occur in
women, usually around age 50. In people with PBC, the immune system attacks and
destroys cells in the livers bile ducts. Like many autoimmune disorders, the causes of
PBC are unknown.
Primary Sclerosing Cholangitis. Primary sclerosing cholangitis (PSC) is a chronic
disease that mostly affects men, usually around age 40. The cause is unknown, but
immune system defects, genetics, and infections may play a role.
Nonalcoholic Fatty Liver Disease (NAFLD) and Nonalcoholic Steatohepatitis
(NASH)
Nonalcoholic fatty liver disease (NAFLD) resembles alcoholic liver disease, but it occurs
in people who do not drink a lot of alcohol. NAFLD is the most common liver disease in
the United States.
NAFLD is actually a progressive spectrum of liver diseases that include:

Nonalcoholic fatty liver (NAFL), or fatty liver, is the earliest stage of NAFLD. It is
marked by the presence of fat in the liver (steatosis), but liver damage has not occurred.
While a fatty liver is not normal, NAFL is not considered a serious condition.
Nonalcoholic steatohepatitis (NASH) is the next stage of NAFLD. NASH is
characterized by liver inflammation and injury, as well as a fatty liver. NASH is
dangerous because it can lead to the scarring of the liver associated with cirrhosis.
NASH is one of the leading causes of cirrhosis.
Cirrhosis is the final irreversible stage of NAFLD.
Obesity and type 2 diabetes are the two main causes of NAFLD. Metabolic syndrome is
another major factor. Metabolic syndrome is a collection of risk factors that include
abdominal obesity, unhealthy blood lipid levels, high blood pressure, and insulin
resistance.
Nonalcoholic fatty liver disease is usually benign and very slowly progressive. But, in
certain patients, it can lead to cirrhosis and eventual liver failure. NAFLD also increases
the risk for heart disease, which is the leading cause of death for these patients.
Hereditary Disorders
Hemochromatosis. Hemochromatosis is a disorder of iron metabolism. This disease
interferes with the way the body normally handles iron. People with hemochromatosis
absorb too much iron from the food they eat. The iron overload accumulates in organs
in the body. When excess iron deposits accumulate in the liver, they can cause
cirrhosis.
Other Hereditary Disorders. Other inherited diseases that can cause cirrhosis include
Wilsons disease (which causes an accumulation of copper in the body), alpha-1
antitrypsin deficiency (a genetic disorder caused by defective production of a particular
enzyme), and glycogen storage diseases (a group of disorders that cause abnormal
amounts of glycogen to be stored in the liver).
Other Causes
Schistosomiasis, a disease caused by a parasite found in the Asia, Africa, and
South America.
Long-term or high-level exposure to certain chemicals and drugs, including
arsenic, methotrexate, toxic doses of vitamin A, and certain prescription medications.
Symptoms
Cirrhosis is divided into two stages: Compensated and decompensated.
Compensated cirrhosis means that the body still functions fairly well despite
scarring of the liver. Many people with compensated cirrhosis experience few or no
symptoms.
Decompensated cirrhosis means that the severe scarring of the liver has
damaged and disrupted essential body functions. Patients with decompensated
cirrhosis develop many serious and life-threatening symptoms and complications.
Early symptoms of compensated cirrhosis may include:
Fatigue and loss of energy
Loss of appetite and weight loss
Nausea or abdominal pain
Spider angiomas may develop on the skin. These are pinhead-sized red spots
from which tiny blood vessels radiate.

As cirrhosis progresses to a decompensated stage, patients may develop the following

symptoms:
Fluid buildup in the legs and feet (edema) and in the abdomen (ascites). (Ascites
is associated with portal hypertension, which is described in the Complications section
of this report.)
Jaundice. This yellowish cast to the skin and eyes occurs because the liver
cannot process bilirubin for elimination from the body.

Itching. Itching (pruritus) develops from buildup of bile products.

The palms of the hands may be reddish and blotchy, a condition known as
palmar erythema
In men, swelling of breasts or shrinkage of the testicles may occur.
Easy bruising and excessive bleeding may occur.
Complications
A damaged liver affects almost every bodily process, including the functions of the
digestive, hormonal, and circulatory systems. Decompensated cirrhosis increases the
risk of serious and potentially life-threatening complications. (Once decompensation
occurs, mortality rates without liver transplantation can be as high as 85% within 5
years.) The most serious complications are those associated with portal hypertension
(increased pressure in the portal vein that carries blood from the intestine to the liver).
They include:
Ascites (fluid buildup in the abdomen)
Variceal hemorrhage (bleeding in the upper stomach and esophagus from
ruptured blood vessels)
Spontaneous bacterial peritonitis is a form of peritonitis (inflammation of the
membrane that lines the abdomen), which is associated with ascites. Other bacterial
infections are also a common complication of cirrhosis.
Hepatic encephalopathy (brain dysfunction). Impaired brain function occurs when
the liver cannot detoxify harmful substances, and can lead to coma.

Liver cancer is a serious long-term risk with cirrhosis. Other complications also occur.
Ascites
Ascites is fluid buildup in the abdominal cavity. It is uncomfortable and can impair
breathing and other functions. Ascites is caused by a combination of portal hypertension
(high pressure in the blood vessels of the liver) and low albumin levels. Albumin is a
protein produced by the liver. Although ascites itself is not fatal, it is a marker for severe
progression.
Hepatorenal syndrome occurs if the kidneys drastically reduce their own blood flow in
response to the altered blood flow in the liver. It is a life-threatening complication of latestage liver disease that occurs in patients with ascites. Symptoms include dark colored
urine and a reduction in volume, yellowish skin, abdominal swelling, mental changes
(such as delirium and confusion), jerking or coarse muscle movement, nausea, and
vomiting.
Variceal Bleeding
One of the most serious consequences of portal hypertension is the development of
varices, veins that enlarge to provide an alternative pathway for blood diverted from the
liver. In most patients, they form in the esophagus. They can also form in the upper
stomach. Varices pose a high risk for rupture and bleeding because they are thinwalled, twisted, and subject to high pressure. Variceal intestinal bleeding is a lifethreatening event. Symptoms include vomiting blood or black and tarry stools.
Spontaneous Bacterial Peritonitis
Spontaneous bacterial peritonitis is a life-threatening bacterial infection of the
membrane that lines the abdomen. The main symptoms include confusion and altered
mental status, fever, chills, and abdominal pain.
Hepatic Encephalopathy
Mental impairment is a common event in advanced cirrhosis. In severe cases, the
disease causes encephalopathy (impaired brain function), with mental symptoms that
range from confusion to coma and death. Hepatic encephalopathy is caused by a
buildup in the blood of harmful intestinal toxins, particularly ammonia, which then
accumulate in the brain. Encephalopathy can be triggered by many different conditions
including internal bleeding, infection, constipation, and dehydration.
Early symptoms of hepatic encephalopathy include confusion, forgetfulness, and trouble
concentrating. Sudden changes in the patient's mental state, including agitation or
confusion, may indicate an emergency condition. Other symptoms include fruitysmelling bad breath and tremor. Late-stage symptoms of encephalopathy are stupor
and eventually coma.
Liver Cancer
People with cirrhosis have an increased risk for hepatocellular carcinoma, a type of liver
cancer. Hepatitis B and C, alcoholism, hemochromatosis, and primary biliary cirrhosis -all causes of cirrhosis -- are some of the major risk factors for liver cancer. Cirrhosis due
to hepatitis C is the leading cause of hepatocellular carcinoma in the United States.
Other Complications
Kidney Failure. Portal hypertension and spontaneous bacterial peritonitis can cause
several secondary complications, including kidney failure. Nonsteroidal antiinflammatory drugs (NSAIDs) -- such as ibuprofen (Advil, Motrin, generic), naproxen
(Aleve, generic), and aspirin -- can also cause kidney failure in patients with cirrhosis.

Osteoporosis. Many patients with cirrhosis develop osteoporosis, a bone-thinning

disease. [For more information, see In-Depth Report #18: Osteoporosis.]

Osteoporosis is a condition characterized by progressive loss of bone density, thinning

of bone tissue, and increased vulnerability to fractures. Osteoporosis may result from
disease, dietary or hormonal deficiency, or advanced age. Regular exercise and vitamin
and mineral supplements may reduce and even reverse loss of bone density.
Insulin Resistance and Type 2 Diabetes. Cirrhosis often causes insulin resistance, a
primary feature in type 2 diabetes. As insulin resistance progresses, it causes excess
sugar (glucose) to build up in the blood, which leads to type 2 diabetes. In turn, type 2
diabetes is also a risk factor for nonalcoholic fatty liver disease, one of the causes of
cirrhosis. [For more information, see In-Depth Report #60: Diabetes type 2.]
Heart Problems. Cirrhosis may increase the risk for heart failure and other
cardiovascular complications.
Diagnosis
A physical examination may reveal the following in a patient with cirrhosis:
The cirrhotic liver is firm and often enlarged in early stages of the disease. The
liver may feel rock-hard. (In advanced stages of cirrhosis, the liver may become small
and shriveled.)
If the abdomen is swollen, the doctor will check for ascites by tapping the flanks
and listening for a dull thud and feeling the abdomen for a shifting wave of fluid.
The doctor will also check for signs of jaundice, muscle wasting, and (in male
patients) breast enlargement.
A patients medical history is another indicator of the risk for cirrhosis. Patients with a
history of alcoholism, hepatitis B or C, or certain other medical conditions are at high
risk.
Other tests (blood tests, imaging tests, liver biopsy) may also be performed. The results
of these tests along with the presence of specific complications (ascites and
encephalopathy) are used for calculating the Child-Pugh Classification. This is a staging

system (A to C) that helps doctors determine the severity of cirrhosis and predict the
development of future complications.
Blood Tests
A patients medical history can reveal risk factors (such as alcoholism) that warrant
screening for conditions such as hepatitis. Blood tests are also performed to measure
liver enzymes associated with liver function. Enzymes known as aminotransferases,
including aspartate (AST) and alanine (ALT), are released when the liver is damaged.
Blood tests may also measure:
Serum albumin concentration. Serum albumin measures the protein in the blood
(low levels indicate poor liver function).
Prothrombin time (PT). The PT test measures in seconds the time it takes for
blood clots to form (the longer it takes the greater the risk for bleeding).
Alkaline phosphatase (ALP). High ALP levels can indicate bile duct blockage.
Bilirubin. One of the most important factors indicative of liver damage is bilirubin,
a red-yellow pigment that is normally metabolized in the liver and then excreted in the
bile. In patients with hepatitis, the liver cannot process bilirubin, and blood levels of this
substance rise, sometimes causing jaundice.
Imaging Tests
Magnetic resonance imaging (MRI), computed tomography (CT), and ultrasound are all
imaging techniques that are useful in detecting and defining the complications of
cirrhosis, such as ascites and hepatocellular carcinoma. These imaging tests can also
provide information on the extent of liver damage.

Click the icon to see an image detailing an MRI scan.

Click the icon to see an image detailing a CT scan.

Liver Biopsy
A liver biopsy is the only definite method for confirming a diagnosis of cirrhosis. It also
helps determine its cause, treatment possibilities, the extent of damage, and the longterm outlook. For example, patients with chronic hepatitis C who show no significant
liver scarring when biopsied may have a low risk for cirrhosis.

A biopsy involves a doctor inserting a thin biopsy needle, guided by ultrasound, to

remove a small sample of liver tissue. Local anesthetic is used to numb the area.
Patients may feel pressure and some dull pain. The procedure takes about 20 minutes
to perform.
The biopsy may be performed using various approaches, including:
Percutaneous Liver Biopsy. This approach uses a needle inserted through the
skin over the liver area to obtain a tissue sample from the liver. Various forms of needles
are used, including those that use suction or those that cut out the tissue. This approach
should not be used in patients with bleeding problems, and it must be used with caution
in patients with ascites or severe obesity.

Click the icon to see an image detailing liver biopsy.

Transjugular Liver Biopsy. This approach uses a catheter (a thin tube) that is
inserted in the jugular vein in the neck and threaded through the hepatic vein (which
leads to the liver). A needle is passed through the tube, and a suction device collects
liver samples. This procedure is risky but may be used for patients with severe ascites.
Laparoscopy. This procedure requires a small abdominal incision through which
the doctor inserts a thin tube that contains small surgical instruments and a tiny camera
to view the surface of the liver. This is generally reserved for staging liver cancer or for
ascites of unknown cause.
Other Tests Used to Detect Complications of Cirrhosis
Endoscopy. Some doctors recommend endoscopy for patients newly diagnosed with
mild-to-moderate cirrhosis in order to screen for esophageal varices. (These are
enlarged veins in the esophagus that increase the risk for bleeding). In this test, a fiberoptic tube is inserted down the throat. The tube contains tiny cameras to view the inside
of the esophagus, where varices are most likely to develop.
Paracentesis. If ascites is present, paracentesis is performed to determine its cause.
This procedure involves using a thin needle to withdraw fluid from the abdomen. The
fluid is tested for different factors to determine the cause of ascites:
Bacteria cultures and white blood cell counts. (These are used to diagnose
infection.)
Protein levels. Low levels of protein in the fluid plus a low white blood cell count
suggest that cirrhosis is the cause of the ascites.
Tests for Liver Cancer. Some doctors will screen patients with cirrhosis every 6 months
to check for the development of liver cancer (hepatocellular carcinoma). To do this, a
doctor will use both a blood test to check for levels of alpha-fetoprotein and an imaging
test (ultrasound, MRI, or CT scan).
Treatment

Cirrhosis is an irreversible condition. Treatment goals are to slow the progression of

liver damage and reduce the risk of further complications. There are currently no drugs
available to treat liver scarring, although researchers are investigating various antifibrotic drugs.
Dietary and Lifestyle Changes
All patients with cirrhosis can benefit from certain types of lifestyle interventions. These
include:
Stop drinking alcohol. It is very important for people with cirrhosis to completely
abstain from alcohol.
Restrict dietary salt. Sodium (salt) can increase fluid buildup in the body. Eating a
variety of foods every day can help you limit the amount of salt you are getting. It is best
to eat fresh vegetables and fruits whenever possible and to avoid eating processed
foods.
Eat a healthy diet. People with cirrhosis are typically malnourished and require
increased calories and nutrients. (Excess protein, however, can trigger hepatic
encephalopathy.) They also need to avoid certain foods, such as raw seafood or
shellfish, which carry risks of blood poisoning (septicemia). A dietician can provide
dietary guidelines.
Get vaccinated. Patients with cirrhosis should ask their doctors which
vaccinations (such as hepatitis A, hepatitis B, influenza, pneumococcal pneumonia) they
need.
Discuss all medications with your doctor. Before you take any medications,
(including nonprescription pain relievers such as acetaminophen), ask your doctor if
they are safe for you. Liver damage affects the metabolism of drugs. Nonsteroidal antiinflammatory drugs (NSAIDs) such as aspirin, ibuprofen, and naproxen should not be
used by patients with cirrhosis as they can trigger bleeding, worsen the condition, and
potentially cause kidney failure.
Inform your doctor of any herbs or supplements you are considering taking.
Certain types of herbal remedies (kava, chaparral, kombucha mushroom, mistletoe,
pennyroyal, and some traditional Chinese herbs) can increase the risk for liver damage.
Although some herbs, such as milk thistle (silymarin) have been studied for possible
beneficial effects on liver disease, there is no scientific evidence to show that they can
help.
Recognizing Signs of Dangerous Complications
Patients with cirrhosis are susceptible to infections and bleeding, both of which can be
life threatening. Contact your doctors office or go to the emergency room if you
experience any of the following symptoms:
Fever (temperature greater than 101 F)
Confusion that is new or suddenly becomes worse
Vomiting more than once a day
Rectal bleeding, black, tarry stools, vomiting blood or dark coffee ground
material, or blood in the urine
Diarrhea
Abdominal or chest pain
Shortness of breath

Abdominal swelling or ascites that is new or suddenly becomes worse

Jaundice (yellowing skin or eyes) that is new or suddenly becomes worse
Treatment of Underlying Conditions
Treatment for cirrhosis depends on the cause of cirrhosis.
Chronic Hepatitis. Many types of antiviral drugs are used to treat chronic hepatitis B,
including pegylated interferon, nucleoside analogs, and nucleotide analogs. Patients
with chronic hepatitis C are treated with combination therapy with pegylated interferon
and ribavarin. In 2011, two new drugs telaprevir (Incivek) and boceprevir (Victrelis)
were approved for use with hepatitis C combination therapy. [For more information,
see In-Depth Report #59: Hepatitis.]
Autoimmune Hepatitis. Autoimmune hepatitis is treated with the corticosteroid
prednisone and also sometimes immunosuppressants, such as azathioprine (Imuran).
Bile Duct Disorders. Ursodeoxycholic acid (Actigall, generic), also known as ursodiol or
UDCA, is used for treating primary biliary cirrhosis but does not slow the progression.
Itching is usually controlled with cholesterol drugs such as cholestyramine (Questran,
generic) and colestipol (Colestid). Antibiotics for infections in the bile ducts and drugs
that quiet the immune system (prednisone, azathioprine, cyclosporine, methotrexate)
may also be used. Several surgical procedures may also be tried to open up the bile
ducts.
Nonalcoholic Fatty Liver Disease (NAFLD) and Nonalcoholic Steatohepatitis
(NASH). Weight reduction through diet and exercise, and diabetes and cholesterol
management are the primary approaches to treating these diseases.
Hemochromatosis. Hemachromatosis is treated with phlebotomy, a procedure that
involves removing about a pint of blood once or twice a week until iron levels are
normal.
Treatment of Complications
Treatment of Ascites
First-line treatment of patients with ascites (fluid accumulation in the abdomen) involves:
Dietary salt restriction (generally less than 1,500 mg/day of sodium)
Drug treatment with diuretics, usually spironolactone (Aldactone, generic) and
furosemide (Lasix, generic).
Complete abstention from alcohol
Fluid restriction is usually not necessary unless sodium levels in the blood are
low.
Treatment for Recurring or Refractory Ascites. Patients with ascites that does not
respond to standard diuretics after a month (refractory ascites) may require procedures
to reduce fluid:
Large-volume paracentesis, (which involves using a thin needle to withdraw fluid
from the abdomen), may be used for ascites refractory to medical treatment or when
complications are present.
Transjugular intrahepatic portosystemic shunt (TIPS) uses a stent placed in veins
in the middle of the liver to keep open a passage connecting the hepatic and portal
veins. This helps reroute blood around the scarred liver. In the procedure, a long needle
is inserted into the jugular vein in the neck and passed down to the hepatic and portal
veins. TIPS is used for patients with ascites unresponsive to treatment and those who

may require a liver transplant. In general, TIPS should be a second-line option for
ascites that does not respond to diuretics.
Treatment of Spontaneous Bacterial Peritonitis
Patients with ascites who have high white blood cell counts should receive intravenous
antibiotic therapy (usually cefotaxime) or oral antibiotic therapy with ofloxacin. Patients
who have had an episode of spontaneous bacterial peritonitis are treated with long-term
antibiotic therapy of norfloxacin (Noroxin) or trimethoprim/sulfamethoxazole (Bactrim,
Septra, generic) to prevent further infection.
Treatment of Hepatorenal Syndrome
Hepatorenal syndrome can occur in patients with ascites. This is a life-threatening
condition in which kidney failure develops because of altered blood flow in the liver.
Patients with hepatorenal syndrome are treated with intravenous infusion of albumin.
Drug therapy includes oral midodrine (ProAmatine, generic) and octreotide
(Sandostatin, generic). Studies suggest that the vasoconstrictor drug terlipressin, given
in combination with albumin, may be helpful for treating hepatorenal syndrome.
Treatment of Hepatic Encephalopathy
The first step in managing encephalopathy (damage to the brain) is to treat any
precipitating cause, such as:
High ammonia levels
Bleeding
Low oxygen
Dehydration
Infection
Use of sedatives
A protein-restricted diet may be used to lower ammonia production. The laxative
lactulose, given as a syrup or enema, is used to empty the bowels and to help improve
mental status. The antibiotic neomycin may be added for patients who do not improve
with lactulose alone. Rifaximin (Xifaxan) is another antibiotic used for treatment of
hepatic encephalopathy.
Treatment of Variceal Bleeding
Primary Prevention. Primary prevention means treating the varices (swollen or
distended veins) before they have bled. Varices that are present in the esophagus,
stomach, or intestines are always at risk of bleeding. Nonselective beta-blockers drugs,
which are commonly used to treat high blood pressure, may be given to prevent
bleeding. Propanolol (Inderal, generic) or nadolol (Corgard, generic) are the standard
beta-blockers used for variceal prevention.
Patients with medium-to-large varices that have not bled may also be treated with a
surgical procedure called endoscopic variceal ligation (EVL). EVL is also called band
ligation. It involves inserting an endoscope or tube down through the esophagus. The
equipment contains microcameras and tiny instruments. Latex bands are wrapped
around the bleeding varices to shut off the blood supply.
Other types of therapies, (such as nitrate drugs, shunts, or sclerotherapy), are generally
not recommended for primary prevention of variceal bleeding.
Treatment. When varices located in the digestive tract begin to bleed (variceal
hemorrhage), it is considered an emergency situation. The first step is to immediately

achieve normal blood clotting (hemostasis) in order to stop the current bleeding
episode. Patients almost always need blood transfusions.
The primary treatment for variceal hemorrhage is drug therapy with ocreotide
(Sandostatin, generic). This drug is given for 3 - 5 days after the bleeding began to
reduce the risk for rebleeding. Endoscopic variceal ligation (described above) is usually
the preferred method. An alternative procedure is endoscopic sclerotherapy. In
endoscopic sclerotherapy, the endoscopic tube is inserted through the mouth and a
sclerosant (a solution that toughens the tissue around the variceal blood vessels) is
injected to stop the bleeding.
If these treatments do not control the bleeding, or bleeding recurs, a transjugular
intrahepatic portosystemic shunt (TIPS) procedure is performed. (For more information
on TIPS, see "Treatment of Ascites" above.) TIPS is not useful as the first choice for
stopping an initial bleeding episode or for preventing rebleeding since it poses a high
risk for encephalopathy.
Another procedure, called balloon tamponade, may be used to temporarily control
bleeding prior to the TIPS procedure. Balloon tamponade is performed only for bleeding
that cannot be controlled by drugs or endoscopy. It involves inserting a tube through the
nose and down through the esophagus until it reaches the upper part of the stomach. A
balloon at the tube's end is inflated and positioned tightly against the esophageal wall. It
is usually deflated in about 24 hours. Balloon tamponade poses a risk for serious
complications, the most dangerous being rupture of the esophagus.
Secondary Prevention. Patients who survive an episode of variceal bleeding need to be
treated with drugs to prevent bleeding recurrence. Patients are prescribed either a
combination of a nitrate drug (such as isosorbide, which is used to treat angina) and a
nonselective beta-blocker (propanolol or nadolol) or a beta-blocker alone. Patients are
also given several sessions of endoscopic variceal ligation over the course of several
months. The TIPS procedure may be recommended for patients who experience
recurrent bleeding despite drug and endoscopic therapy.
Liver Transplantation
When cirrhosis progresses to end-stage liver disease, patients may be candidates for
liver transplantation. Patients with liver cancer that has not spread beyond the liver are
also candidates for transplant.
Current 5-year survival rates after liver transplantation are about 75%. Patients report
improved quality of life and mental functioning after liver transplantation. Patients should
seek medical centers that perform more than 50 transplants per year and produce
better-than-average results.
A scoring system called Model for End-Stage Liver Disease (MELD) is used to
determine which patients are most in need of a donor liver. A MELD score predicts 3month survival based on laboratory tests of creatinine, bilirubin, and blood-clotting time.
Priority is given to patients who are most likely to die without a liver transplant.
Unfortunately, there are many more patients waiting for liver transplants than there are
available organs. Patients may also want to consider living-donor liver transplantation.
In living-donor transplantation, surgeons replace the patients diseased liver with a part
of the liver taken from a donor. The donors liver regenerates to full size within a few
weeks of surgery, and the recipients liver also regrows. This procedure produces
excellent results for patients, but there are some risks for the donor.

Transplantation surgery generally takes 4 - 12 hours to perform, and patients stay in the
hospital for up to 3 weeks after the surgery. Most patients return to normal or nearnormal activities 6 - 12 months following the transplant. For the rest of their lives,
patients need to take immunosuppressive medication to prevent rejection.
Liver Transplantation in Patients with Viral Hepatitis. One of the primary problems with
performing liver transplantation in patients with hepatitis is recurrence of the virus after
transplantation. Recurrence typically occurs with hepatitis C. Viral recurrence can also
occur with hepatitis B. HepaGam B is an immune globulin preparation approved to
prevent recurrence of hepatitis B after transplantation. Patients need to receive periodic
HepaGam B injections on a lifelong basis.
Liver Transplantation for Patients with Primary Biliary Cirrhosis. Patients who require
transplantation for primary biliary cirrhosis are those who develop major complications
of portal hypertension and liver failure or who have poor quality of life and short survival
without the procedure. Survival rates after transplantation are excellent.
Liver Transplantation for Patients with Autoimmune Hepatitis. The outlook is also good
for patients who have autoimmune hepatitis who require a transplant. Survival rates are
about 90% after 1 year, and 70 - 80% after 5 years. Rejection usually occurs in those
patients whose immune systems are very compromised.
Liver Transplantation for Patients with Alcoholism. Liver transplantation is generally not
recommended for patients with active alcohol or drug abuse addictions.

Click the icon to see an illustrated series detailing a liver transplant.

Resources
www2.niddk.nih.gov -- National Institute of Diabetes and Digestive and Kidney
Diseases
www.aasld.org -- American Association for the Study of Liver Diseases
www.liverfoundation.org -- American Liver Foundation
www.gastro.org -- American Gastrointestinal Association
www.cdc.gov/ncidod/diseases/hepatitis -- Centers for Disease Control and
Prevention, Hepatitis
www.hepfi.org -- Hepatitis Foundation International
www.unos.org -- United Network for Organ Sharing
www.organdonor.gov -- US government organ donor site
References
Berg CL, Gillespie BW, Merion RM, Brown RS Jr, Abecassis MM, Trotter JF, et al
Improvement in survival associated with adult-to-adult living donor liver
transplantation. Gastroenterology. 2007 Dec;133(6):1806-13. Epub 2007 Sep 14.

Brown RS Jr. Live donors in liver transplantation. Gastroenterology. 2008

May;134(6):1802-13.
Crdenas A, Gins P. Management of patients with cirrhosis awaiting liver
transplantation. Gut. 2011 Mar;60(3):412-21. Epub 2010 Dec 30.
Chalasani N, Younossi Z, Lavine JE, Diehl AM, Brunt EM, Cusi K, et al. The diagnosis
and management of non-alcoholic fatty liver disease: practice Guideline by the
American Association for the Study of Liver Diseases, American College of
Gastroenterology, and the American Gastroenterological Association. Hepatology. 2012
Jun;55(6):2005-23.
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al.
Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010
Feb;51(2):660-78.
Chandok N, Watt KD. Pain management in the cirrhotic patient: the clinical
challenge. Mayo Clin Proc. 2010 May;85(5):451-8. Epub 2010 Mar 31.
Garcia-Tsao G, Bosch J. Management of varices and variceal hemorrhage in
cirrhosis. N Engl J Med. 2010 Mar 4;362(9):823-32.
Garcia-Tsao G, Lim JK; Members of Veterans Affairs Hepatitis C Resource Center
Program. Management and treatment of patients with cirrhosis and portal hypertension:
recommendations from the Department of Veterans Affairs Hepatitis C Resource Center
Program and the National Hepatitis C Program. Am J Gastroenterol. 2009
Jul;104(7):1802-29. Epub 2009 May 19
Garcia-Tsao G, Sanyal AJ, Grace ND, Carey WD; Practice Guidelines Committee of
American Association for Study of Liver Diseases; Practice Parameters Committee of
American College of Gastroenterology. Prevention and management of
gastroesophageal varices and variceal hemorrhage in cirrhosis. Am J Gastroenterol.
2007 Sep;102(9):2086-102.
Gins P, Schrier RW. Renal failure in cirrhosis. N Engl J Med. 2009 Sep
24;361(13):1279-90.
Gonzalez R, Zamora J, Gomez-Camarero J, Molinero LM, Baares R, Albillos A. Metaanalysis: Combination endoscopic and drug therapy to prevent variceal rebleeding in
cirrhosis. Ann Intern Med. 2008 Jul 15;149(2):109-22.
Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. N Engl J
Med. 2007 Oct 11;357(15):1524-9.
Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ; American
Association for Study of Liver Diseases. Primary biliary cirrhosis. Hepatology. 2009
Jul;50(1):291-308.
Marchesini G, Moscatiello S, Di Domizio S, Forlani G. Obesity-associated liver
disease. J Clin Endocrinol Metab. 2008 Nov;93(11 Suppl 1):S74-80.
O'Leary JG, Lepe R, Davis GL. Indications for liver transplantation. Gastroenterology.
2008 May;134(6):1764-76.
Parikh S, Hyman D. Hepatocellular cancer: a guide for the internist. Am J Med. 2007
Mar;120(3):194-202.
Parikh S, Shah R, Kapoor P. Portal vein thrombosis. Am J Med. 2010 Feb;123(2):111-9.
Runyon BA; AASLD Practice Guidelines Committee. Management of adult patients with
ascites due to cirrhosis: an update. Hepatology. 2009 Jun;49(6):2087-107.

Salerno F, Camm C, Enea M, Rssle M, Wong F. Transjugular intrahepatic

portosystemic shunt for refractory ascites: a meta-analysis of individual patient
data. Gastroenterology. 2007 Sep;133(3):825-34. Epub 2007 Jun 20.
Schuppan D, Afdhal NH. Liver cirrhosis. Lancet. 2008 Mar 8;371(9615):838-51.
Udell JA, Wang CS, Tinmouth J, FitzGerald JM, Ayas NT, Simel DL, et al. Does this
patient with liver disease have cirrhosis? JAMA. 2012 Feb 22;307(8):832-42.

Source: Cirrhosis | University of Maryland Medical

Center http://umm.edu/health/medical/reports/articles/cirrhosis#ixzz3XIcrODL1
University of Maryland Medical Center
Follow us: @UMMC on Twitter | MedCenter on Facebook

Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical

School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD,
MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
Source: Cirrhosis | University of Maryland Medical
Center http://umm.edu/health/medical/reports/articles/cirrhosis#ixzz3XId17oBs
University of Maryland Medical Center
Follow us: @UMMC on Twitter | MedCenter on Facebook