What is cirrhosis?
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. There are several functions of the liver that are impaired by the scarring that occurs in cirrhosis.
- Controlling infections
- Removal of bacteria and toxins from the blood
- Processing nutrients, hormones and drugs
- Making proteins that regulate blood clotting
- Bile production 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.
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: Heavy alcohol use over several years can cause chronic injury to the liver. The amount of alcohol including beer and wine it takes to damage the liver varies greatly from person to person. Consuming 2 to 3 drinks per day for women and 3 to 4 drinks per day for men 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. Please see section titled Hepatitis C for more information.
- 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. Please see section titled Hepatitis B for more information.
- Nonalcoholic fatty liver disease (NAFLD): Fat can build up in the liver and eventually cause 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. 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.
- 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 signs and 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 signs and symptoms:
- Weakness
- Fatigue
- Loss of appetite
- Nausea/vomiting
- Weight loss
- Abdominal pain and bloating when fluid accumulates in the abdomen (ascites). Ascites can lead to bacterial peritonitis, a serious infection.
- Itching
- Spiderlike blood vessels on the skin
- Leg swelling with fluid (edema)
- 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
- Easy bruising and bleeding including nosebleeds
- Medication sensitivity: Cirrhosis slows the liver’s ability to filter medications from the blood causing medications to act longer than expected and build up in the body
- Medicines may not break down as quickly
- Hepatic encephalopathy: Confusion, lethargy and trouble concentrating due to toxins in the blood
- Portal hypertension: Normally, blood from the intestines and spleen is carried to the liver through the portal vein. Cirrhosis slows the normal flow of blood, increasing the pressure in the portal vein.
- 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.
- Enlarged veins in the esophagus and stomach (varices). These varices can lead to serious sudden bleeding causing vomiting of blood or passage of blood in a bowel movement. This complication of cirrhosis requires immediate medical attention.
- Kidney disease and/or failure
- Increased risk of liver cancer
- Insulin resistance and type 2 diabetes: Insulin is 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.
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. For example, on abdominal examination, the liver may feel hard or enlarged with signs of ascites. Blood tests can 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 several imaging modalities are available including computerized tomography (CT) scan, ultrasound, and magnetic resonance imaging (MRI).
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 by an interventional radiologist with a needle inserted 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: Malnutrition is common in people with cirrhosis so a healthy diet is important in all stages of the disease. 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.
- Avoiding alcohol and other substances: People with cirrhosis are strongly encouraged to avoid any alcohol or illicit substances, as both will cause more liver damage. Because many vitamins and medications, both prescription and over-the-counter, can affect liver function, Dr. Makhani should be consulted before taking them.
Treatment for cirrhosis also addresses specific complications.
- Edema and ascites: Diuretics are 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.
- Portal Hypertension: Beta-blockers can lower the pressure in the varices and reduce the risk of bleeding. Gastrointestinal bleeding requires an urgent upper endoscopy to evaluate for esophageal varices. Dr. Makhani 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: Treated by cleansing the bowel with lactulose, a laxative given orally or in enemas. An antibiotic called Rifaximin may be added to the treatment if necessary.
- Kidney failure: Also called hepatorenal syndrome may cause patients to 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.
How can cirrhosis be prevented if a person already has liver disease?
Many of the causes of cirrhosis are treatable. Early treatment may prevent cirrhosis. Some management tips include:
- Weight loss and keeping weight in the normal range. Being overweight can make several liver diseases worse.
- Avoiding alcohol intake. Alcohol can harm liver cells. Drinking large amounts of alcohol over many years is one of the major causes of cirrhosis.
- Avoiding illegal drugs, which can increase chances of getting Hepatitis B or Hepatitis C.
- Treatments for hepatitis B, C, and D are available. If treatment is started, it is very important to carefully follow treatment directions.
- Taking the appropriate medications for those people with liver disease from autoimmune hepatitis.
- Vaccinations against Hepatitis A and Hepatitis B. Although Hepatitis A does not cause cirrhosis, it can damage the liver
- Yearly flu vaccination and age appropriate pneumonia vaccination
- Avoidance of raw oysters or other raw shellfish. Raw shellfish can have bacteria that cause severe infections in people with cirrhosis.
- Routine monitoring with imaging and blood test to detect early signs of liver cancer.
When is a liver transplant indicated for cirrhosis?
Liver transplantation 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.