Cirrhosis is a disease in which normal liver cells are damaged and replaced with scar tissue, disrupting liver function. It may be caused by direct injury to liver cells, or by indirect injury, such as inflammation or obstruction of the ducts that drain bile from the liver.
The liver is the largest internal organ in the body. It is located mainly in the upper right side of the abdomen, situated mostly under the lower ribs. It weighs about 3 pounds and is roughly the size of a football.
Most of the blood that leaves the stomach and intestines passes through the liver.
The liver performs hundreds of functions necessary for survival, including:
Producing blood proteins that regulate blood clotting
Producing bile and enzymes necessary for digestion
Storing energy to fuel muscles
Helping to maintain glucose (blood sugar) concentration
Helping to regulate hormones
Removing toxins from drugs and poisons, including alcohol
Under normal circumstances, the liver has the ability to heal itself by repairing or replacing damaged cells. With cirrhosis, the healing process is altered. Special cells, called stellate cells, increase dramatically in size and number and cause excess scar tissue to form. When scar tissue is present, it decreases the amount of normal tissue and interferes with normal blood flow through the liver which, in turn, causes liver function to deteriorate. Cirrhosis usually progresses slowly over time, but the damage done to the liver is irreversible.
Because cirrhosis impairs liver function, it can lead to many serious complications. They include:
Portal hypertension. Blood from the intestines, spleen and pancreas enter the liver through a large blood vessel called the portal vein. When scar tissue in the liver impedes blood flow it can result in increased pressure in the vein.
Varices. Stretched and dilated veins. When blood flow in the portal vein is blocked, it can lead to abnormally increased blood flow in nearby blood vessels, particularly in the stomach and esophagus. Because the walls of the blood vessels are thin, this can lead to bleeding. Massive bleeding in the stomach or esophagus is a life-threatening emergency that requires immediate medical treatment.
Fluid retention. Cirrhosis can cause large amounts of fluid to accumulate in the legs (edema) or abdominal cavity (ascites). Ascites can cause discomfort and may interfere with breathing or become infected (spontaneous bacterial peritonitis).
Bruising and bleeding. Liver damage interferes with the liver’s absorption of vitamin K and the production of proteins that help the blood clot. As a result, patients may bruise easier and bleed more frequently. Bleeding is particularly common in the gastrointestinal tract.
Jaundice. When the liver is not able to remove bilirubin (substance formed from the breakdown of red blood cells that gives bile its color), jaundice may occur. This causes the skin and whites of the eyes to turn yellow.
Itching. Bile salts deposited in the skin can cause itching.
Hepatic encephalopathy. Abnormal brain function that occurs when toxins from the liver enter the blood. Symptoms include forgetfulness, confusion, mood changes and, in advanced cases, delirium and coma.
Infection. The decreased immune system function from liver damage may lead to increased bacterial infections.
Osteoporosis. Decreased bone mass and density. Cirrhosis interferes with the liver’s ability to produce vitamin D and calcium, which promote bone growth, density and health.
Liver cancer. Cirrhosis increases the risk of developing liver cancer.
Liver failure. This occurs when damage to the liver becomes so extensive that the liver cannot function.
Kidney failure. Cirrhosis may cause the kidneys to function improperly or to fail (hepatorenal syndrome).
Cirrhosis is the 12th leading cause of death by disease in the United States, and results in about 26,000 deaths each year, according to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK).
Risk factors and causes of cirrhosis
In the United States, the leading cause of cirrhosis is chronic alcoholism. Alcoholic cirrhosis usually occurs after a decade or more of heavy drinking, although the amount of alcohol that results in damage to the liver varies among individuals. Some people who drink occasionally (social drinkers) may also develop cirrhosis. The liver breaks down alcohol into highly toxic chemicals, some of which trigger inflammation that eventually destroys liver cells.
The second most common cause of cirrhosis is chronic hepatitis B and hepatitis C, both of which cause inflammation of the liver that can lead to cirrhosis. Hepatitis B is most likely the most common cause of cirrhosis worldwide, but is less common in the United States. Hepatitis C is a major cause of cirrhosis in the United States. Infection with hepatitis B or C slowly damages the liver over time. About one in five people with chronic hepatitis C eventually develops cirrhosis.
Other, less common, causes of cirrhosis include:
A liver disease in which the immune system attacks liver cells. This causes inflammation of the liver, which may be severe and chronic and result in cirrhosis.
Nonalcoholic steatohepatitis (NASH)
Inflammation and excessive fat buildup in the liver that is not associated with alcohol use. This can lead to inflammation and the development of scar tissue.
These include diseases that cause high levels of certain minerals to accumulate in the liver, such as Wilson's disease (which causes copper buildup) or hemochromatosis (which causes iron buildup). Cystic fibrosis (a chronic disease characterized by the buildup of thick sticky mucus in the lungs and digestive tract), glycogen storage diseases (which hinder the body's use of sugar) and and alpha 1-antitrypsin deficiency (a disorder in which the liver produces an abnormal amount of a protective protein) can also lead to cirrhosis.
Blocked or inflamed bile ducts
Certain diseases, such as primary biliary cirrhosis and primary sclerosing cholangitis, can cause bile ducts to become inflamed, blocked or scarred. Bile ducts transport bile (which is produced in the liver) to the gallbladder and small intestine. There is chronic inflammation and eventual scarring of the biliary system with subsequent liver cell damage and cirrhosis. Primary sclerosing cholangitis can also lead to bile duct cancer.
Infection with schistosomia, a parasite that occurs mainly in tropical countries, may lead to liver damage and result in cirrhosis.
This type of heart failure can lead to liver congestion or damage.
Prolonged exposure to toxins
People who are exposed to environmental toxins, such as arsenic, face a higher risk of developing cirrhosis.
In some cases, the cause of cirrhosis cannot be identified.
In general, men develop cirrhosis more often than women. However, women who drink heavily have a higher risk for cirrhosis than men.
Signs and symptoms of cirrhosis
Cirrhosis takes years to develop. In the early stages of cirrhosis, many people experience no symptoms. However, as the disease progresses and liver function deteriorates, symptoms may develop.
The most common signs and symptoms include:
Loss of appetite
Fatigue or exhaustion
Loss of interest in sex
Evidence of cirrhosis may not develop until a patient experiences complications of the disease.
When this happens, signs and symptoms that may be present include:
Spider-like veins (spider angiomas) that develop under the skin
Abdominal pain from an enlarged liver
Yellowing of the skin and whites of the eyes (jaundice)
Dark, cola-colored urine
Fluid in the abdominal cavity (ascites)
Swelling of the legs and feet (edema)
Vomiting of blood
Increased sensitivity to drugs
Itching of the hands and feet that spreads to other parts of the body
Mental confusion, such as forgetfulness or difficulty concentrating, or brain damage (encephalopathy)
Abnormal nerve function (peripheral neuropathy)
Enlarged breasts in men (gynecomastia)
Shrinking (atrophy) of testicles in men
Excessive or prolonged bleeding
Esophageal vein bleeding
Diagnosis methods for cirrhosis
Cirrhosis may be diagnosed by a physician during a physical examination that includes a medical history and list of medications. A physician can sometimes identify early stages of cirrhosis by gently pressing the abdomen to determine whether the liver is enlarged and firm. During later stages of the disease the liver shrinks, causing an enlarged spleen which a physician may also identify by gently touching the abdomen.
If a patient is suspected to have cirrhosis, blood tests may be performed. These can detect changes in the body that occur as a result of cirrhosis. Tests include:
Patients with cirrhosis are often anemic and also tend to have larger-than-normal spleens, which leads to abnormally low platelet counts. The loss of functioning liver leads to less clotting protein production and abnormal coagulation labs.
Liver function test
When a liver is damaged, it releases enzymes, which may be detected with a blood test.
Liver function test
When a liver is damaged, it releases enzymes, which may be detected with a blood test.
Bilirubin (substance formed from the breakdown of red blood cells that gives bile its color) is processed in the liver and excreted in urine. When the liver is damaged, it cannot process bilirubin, leading to high levels of bilirubin in the blood.
Imaging tests that detect liver damage may also be performed. They include:
An imaging technology that uses sound waves to produce images of the shape and outline of various tissues and organs of the body.
CAT scan (computed axial tomography)
A test that allows for multiple x-rays to be taken from different angles around the patient. It creates images of organs and bones within the body.
MRI (magnetic resonance imaging)
Safe and noninvasive or minimally invasive imaging test that can help physicians diagnose diseases of numerous organs and vessels. It uses powerful magnets to produce images on a computer screen and film.
A radioactive substance (radioisotope) that highlights the liver is injected into a vein. After the liver absorbs the substance, the liver is scanned and images are displayed on a computer screen.
A laparoscope (thin, lighted tube with a tiny video camera) is inserted into the abdomen through a small incision. Images are displayed on a computer screen.
A liver biopsy is often performed to confirm a diagnosis of cirrhosis. During this procedure, a needle is inserted into the abdomen and a tiny sample of liver tissue is removed. The tissue is examined under a microscope for the presence of scarring or other signs of disease.
Tests may also be performed to screen for complications of cirrhosis, such as a CAT scan, upper endoscopy or capsule endoscopy for esophageal varices.
Treatment options for cirrhosis
There is no cure for cirrhosis. The liver is capable of repairing some damage, but it is limited. The focus of treatment is to prevent or delay further progression of the disease and to reduce complications. This may involve treating the underlying cause of the disease.
All patients, including those with cirrhosis caused by alcohol abuse, must stop drinking alcohol. Some patients require assistance to help them quit, which may involve a chemical dependency evaluation, intervention, counseling, support groups, outpatient treatment program or inpatient residential program. Other drugs that may be related to liver damage should also be avoided. These include acetaminophen, some other over-the-counter drugs and certain vitamin supplements.
Patients with cirrhosis, regardless of the cause of the disease, must follow a healthy diet because the liver requires nutrients to heal. In addition, patients should avoid eating raw seafood because of the potential presence of bacteria. A diet that restricts salt may also be recommended because of the potential for swelling (edema) and fluid in the abdomen (ascites).
Patients with cirrhosis caused by hepatitis may be treated with medication, such as interferon for viral hepatitis or corticosteroids for autoimmune hepatitis.
Treatment may also involve treating complications of the disease, including:
Portal hypertension. Medications that lower blood pressure, such as beta blockers, may be prescribed to lower pressure in the portal vein (large, short vein that carries nutrient-rich blood from the intestines and spleen to the liver).
Bleeding blood vessels. Medications may be prescribed to reduce bleeding from blood vessels. Patients may also undergo medical procedures to stop bleeding. For example, a procedure called an endoscopic variceal band ligation is used to treat bleeding in the esophagus. During this procedure, latex bands are used to stop the blood supply to bleeding vessels.
Fluid retention. Patients may be treated with medications called diuretics to reduce fluid buildup in the legs or abdomen. Some patients require fluid to be removed from the abdominal cavity with a needle using local anesthetic. Patients may require antibiotics to lessen the possibility of bacterial peritonitis.
Itching. Patients may be treated with antihistamines to help stop itching.
Hepatic encephalopathy. Patients may be treated with lactulose, a medication that helps lower blood ammonia, or antibiotics, which reduce the amount of ammonia-producing bacteria in the intestines.
When the liver is so severely damaged that it does not function, patients need a liver transplant. This involves removing the damaged liver and replacing it with one from an organ donor. Though liver transplantation is successful in a large number of patients, the number of patients who need a new liver is much larger than the number of donated organs. In addition, newer technologies, such as living donors who donate a portion of their liver to another person, are being performed. These transplants may become increasingly available to people seeking a liver.
Researchers are studying potential alternatives to a liver transplant, such as an experimental drug that has treated cirrhosis in mice.
Prevention methods for cirrhosis
Some cases of cirrhosis cannot be prevented. However, many cases may be prevented by:
Avoiding alcohol or drinking in moderation. Alcohol is the leading cause of cirrhosis in the United States. Those who avoid alcohol, particularly those with a family history of alcoholism, may prevent cirrhosis.
Protecting against hepatitis B and hepatitis C. The best way to prevent contracting hepatitis B is to receive a vaccination for it. There is no vaccination for hepatitis C, but people can reduce the risk of contracting it by using a condom during sexual intercourse. About half of new cases of hepatitis C are caused by contaminated drug paraphernalia, so drug users should not share needles or other drug paraphernalia.
Avoiding excessive dietary fat and obesity. On rare occasions, fat and obesity can result in an inflammatory change in the liver and cirrhosis can develop.
Questions for your doctor regarding cirrhosis
Preparing questions in advance can help patients have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions about cirrhosis:
Am I at risk for cirrhosis? What steps can I take to reduce this risk?
What makes you suspect that cirrhosis is causing my symptoms?
What tests do you recommend to confirm this diagnosis? What are the benefits and risks?
Is a liver biopsy necessary? How should I prepare? What type of anesthesia is used?
What is causing or contributing to my cirrhosis?
What dietary and other lifestyle changes do you recommend for me?
How should my cirrhosis be treated?
Are there any over-the-counter medications or products that I should avoid?
Could any of my current prescription medications be of concern?
Will I need a liver transplant?
Maintain as active a life as possible.
Elevate swollen feet and legs when resting.
In the early stages, eat a well-balanced diet that is high in carbohydrates, high in protein and low in salt.
Late stages may require protein reduction.
Vitamin and mineral supplements may be necessary.
Don't drink alcohol.
What might complicate it?
Almost all forms of cirrhosis are associated with portal hypertension, esophageal bleeding, enlarged spleen, fluid retention (ascites and edema), and coma. Other complications may include portal vein thrombosis (blood clot formation), the development of liver tumors, altered drug metabolism, spontaneous bacterial peritonitis in which fluid accumulating within the abdomen becomes infected, and hepatic encephalopathy in which the brain is poisoned by high blood levels of certain metabolic byproducts.
Differential diagnoses include other causes of liver cell injury, idiopathic portal hypertension, schistosomiasis, and congenital hepatic fibrosis.
Gastroenterologist, pathologist, and general surgeon.
Notify your physician if
You or a family member has symptoms of cirrhosis.
The following occur during treatment:
Vomiting blood or passing black stool.
Mental confusion or coma.
Fever or other signs of infection (redness, swelling, tenderness or pain).