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Updated 13 February 2013

Cirrhosis of the liver

The liver is the largest organ in the body and is essential in keeping the body functioning properly.

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Summary

  • In cirrhosis damaged and dead liver cells are replaced by fibrous tissue, which leads to fibrosis
  • Cirrhosis is the eighth leading cause of death by disease
  • In South Africa, chronic alcoholism is the most common cause
  • Cirrhosis is irreversible

Description

The liver is the largest organ in the body and is essential in keeping the body functioning properly. It removes or neutralizes poisons from the blood, produces immune agents to control infection, and removes germs and bacteria from the blood. It makes proteins that regulate blood clotting and produces bile to help absorb fats and fat-soluble vitamins. You cannot live without a functioning liver.

In cirrhosis, damaged and dead liver cells are replaced by fibrous tissue, which leads to fibrosis (scarring). Fibrous tissue replaces normal, healthy tissue. The liver cells regenerate in an abnormal pattern primarily forming nodules that are surrounded by fibrous tissue. Grossly abnormal liver architecture eventually ensues and blocks the flow of blood through the organ thus preventing it from performing the essential functions.

Cirrhosis is the eighth leading cause of death by disease, killing about 25 000 people each year. Also, the cost of cirrhosis in terms of human suffering, hospital costs, and lost productivity is high.

Cause

Cirrhosis has many causes. In South Africa, chronic alcoholism is the most common cause.

Alcoholic liver disease

To many people, cirrhosis of the liver is synonymous with chronic alcoholism, but in fact, alcoholism is only one of the causes. Alcoholic cirrhosis usually develops after more than a decade of heavy drinking. The amount of alcohol that can injure the liver varies greatly from person to person. In women, as few as two to three drinks (eg. 40 gram) per day have been linked with cirrhosis and in men, as few as three to four drinks (eg. 80 gram) per day. Sustained consumption of these quantities over a period of a decade, will lead to cirrhosis in susceptible persons. Alcohol seems to injure the liver by blocking the normal metabolism of protein, fats and carbohydrates.

Chronic hepatitis B, C and D

The hepatitis B virus is probably the most common cause of cirrhosis worldwide. Hepatitis B, like hepatitis C, causes liver inflammation and injury that over several decades can lead to cirrhosis. The hepatitis D virus is another virus that infects the liver, but mostly only in people who already have hepatitis B.

Autoimmune hepatitis

This type of hepatitis is caused by a problem with the immune system.

Inherited diseases

Alpha-1 antitrypsin deficiency, hemochromatosis, Wilson's disease, galactosemia, and glycogen storage diseases are among the inherited diseases that interfere with the way the liver produces, processes, and stores enzymes, proteins, metals, and other substances the body needs to function properly.

Non-alcoholic steatohepatitis (NASH)

In NASH, fat builds up in the liver and eventually causes scar tissue. This type of hepatitis appears to be associated with diabetes, protein malnutrition, obesity, coronary artery disease and corticosteroid treatment.

Blocked bile ducts

When the ducts that carry bile out of the liver are blocked, bile backs up and damages liver tissue. In babies, blocked bile ducts are most commonly caused by biliary atresia, a disease in which the bile ducts are absent or injured. In adults, the most common cause is primary biliary cirrhosis, a disease in which the ducts become inflamed, blocked and scarred. Primary biliary cirrhosis is relatively rare in Africa and most of the patients seen here have come from Europe and particulary from the UK. Secondary biliary cirrhosis can happen after gallbladder surgery, if the ducts are inadvertently tied off or injured.

Drugs, toxins and infections

Severe reactions to prescription drugs, prolonged exposure to environmental toxins e.g. organic solvents (dry cleaners, spray painters, refinery workers) or toxins (chemical industry, farm workers). The parasitic infection schistosomiasis, and repeated bouts of heart failure with liver congestion may each lead to cirrhosis.

Symptoms

Many people with cirrhosis have no symptoms in the early stages of the disease. However, as scar tissue replaces healthy cells, liver function starts to fail and a person may experience the following non specific symptoms: exhaustion, fatigue, loss of appetite, nausea, weakness and weight loss.

As the disease progresses, complications may develop. In some people, these may be the first signs of the disease.

Prevalence

Liver disease has important geographic variations in South Africa. It has been shown that South Africans brought up in the rural regions of Northern Province, Mpumalanga and KwaZulu-Natal are at higher risk of contracting hepatitis B virus in the first six years of life, thus leading more often to eventual cirrhosis.

Course

Loss of liver function affects the body in many ways.

The following are common problems, or complications, caused by cirrhosis.

  • Oedema and ascites - When the liver loses its ability to make the protein albumin, water accumulates in the leg (oedema) and abdomen (ascites).
  • Bruising and bleeding - When the liver slows or stops production of the proteins needed for blood clotting, a person will bruise or bleed easily.
  • Jaundice - Jaundice is a yellowing of the skin and eyes that occurs when the diseased liver does not absorb enough bilirubin.
  • Itching - Bile products deposited in the skin may cause intense itching.
  • Gallstones - If cirrhosis prevents bile from reaching the gallbladder, a person may develop gallstones.
  • Toxins in the blood or brain - A damaged liver cannot remove toxins from the blood, causing them to accumulate in the blood and eventually the brain. These toxins can dull mental functioning and cause personality changes, coma and even death. Signs of the build-up of toxins in the brain include neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, or changes in sleep habits.
  • Sensitivity to medication - Cirrhosis slows the liver's ability to filter medications from the blood. Because the liver does not remove drugs from the blood at the usual rate, they act longer than expected and build up in the body. People with cirrhosis often are very sensitive to medications and their side effects.
  • 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 through the portal vein, which increases the pressure inside it. This condition is called portal hypertension.
  • Varices - When bloodflow through the portal vein slows, blood from the intestines and spleen backs up into blood vessels in the stomach and oesophagus. These blood vessels may become enlarged because they are not meant to carry this much blood. The enlarged blood vessels, called varices, have thin walls and carry high pressure, and thus are more likely to burst. If they do burst, the result is a serious bleeding problem in the upper stomach or oesophagus that requires immediate medical attention.
  • Problems in other organs - Cirrhosis can cause immune system dysfunction, leading to infection. Ascites (fluid) in the abdomen may become infected with bacteria normally present in the intestines, and cirrhosis can also lead to kidney dysfunction and failure.

Diagnosis

The doctor may diagnose cirrhosis on the basis of symptoms, laboratory tests, the patient's medical history, and a physical examination. For example, during a physical examination, the doctor may notice that the liver feels harder or larger than usual and order blood tests that can show whether liver disease is present.

The doctor may order a computerized axial tomography (CAT) scan, ultrasound, or a scan of the liver using a radioisotope (a harmless radioactive substance that highlights the liver). Or a laparoscopic procedure, where an instrument is inserted through the abdomen relaying pictures back to a computer screen. At the same time a liver biopsy may be performed, which will confirm the diagnosis. With a biopsy, the doctor uses a needle to take a small sample of tissue from the liver, and then examines it for scarring or other signs of disease.

Treatment

Home

In all cases, regardless of the cause, following a healthy diet and avoiding alcohol are essential. The body needs all the nutrients it can get, and alcohol will only lead to more liver damage.

Medication

Cirrhosis of the liver is irreversible but treatment of the underlying liver disease may slow or stop the progression. Treatment depends on the cause of cirrhosis and any complications a person is experiencing. For example, cirrhosis caused by alcohol abuse is treated by abstaining from alcohol. Treatment for hepatitis-related cirrhosis involves medications used to treat the different types of hepatitis, such as interferon for viral hepatitis and corticosteroids for autoimmune hepatitis. Cirrhosis caused by Wilson's disease, in which copper builds up in organs, is treated with medications to remove the copper. These are just a few examples. Treatment for cirrhosis resulting from other diseases will depend on the underlying cause.

Treatment will also include remedies for complications. For example, for ascites and oedema, the doctor may recommend a low-sodium diet or the use of diuretics, which are drugs that remove fluid from the body. Antibiotics will be prescribed for infections, and various medications can help with itching. Protein causes toxins to form in the digestive tract, so eating less protein will help decrease the buildup of toxins in the blood and brain. The doctor may also prescribe laxatives to help absorb the toxins and remove them from the intestines.

For portal hypertension, the doctor may prescribe blood pressure medication such as a beta-blocker. If the patient bleeds from the varices of the stomach or oesophagus, the doctor can inject these veins with a sclerosing agent administered through a flexible tube (endoscope) that is inserted through the mouth and oesophagus. Alternatively, he can perform a rubber-band ligation, which uses a special device to compress the varices and stop the bleeding.

Surgery

When the complications cannot be controlled or when the liver becomes so damaged from scarring that it completely stops functioning, a liver transplant is necessary. In liver transplantation surgery, a diseased liver is removed and replaced with a healthy one from an organ donor. About 80 to 90 percent of people survive liver transplantation. Survival rates have improved over the past several years because of drugs such as cyclosporine and azathioprine, which suppress the immune system and keep it from attacking and damaging the new liver.

Prevention

Due to the many causes of cirrhosis, prevention is not in all cases possible.

Reviewed by Prof Don du Toit (M.B.Ch.B) (D.Phil.) (Ph.D) (FCS) (FRCS)

 
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