Cirrhosis is the term used to describe a diseased liver that has been severely scarred, usually due to many years of continuous injury. The most common causes include longstanding alcohol abuse, chronic hepatitis B or hepatitis C, and nonalcoholic steatohepatitis (a condition in which fat builds up in the liver and the liver becomes inflamed). Although cirrhosis cannot be cured, there are a number of things you can do to prevent the disease from worsening.
This topic discusses the symptoms, diagnosis, and treatment of cirrhosis. Related topics are discussed separately. (See "Patient information: Hepatitis B (Beyond the Basics)" and "Patient information: Hepatitis C (Beyond the Basics)" and "Patient information: Alcohol use — when is drinking a problem? (Beyond the Basics)".)
The liver is a large organ (weighing about three pounds) that is located in the right upper abdomen beneath the rib cage (figure 1). It performs many functions that are essential to life.
The liver is able to repair itself when it has been injured. However, the process of healing involves the creation of scar tissue. Thus, repeated or continuous injury to the liver (such as occurs with heavy alcohol use) can cause significant scarring in the liver. The body is able to tolerate a partially scarred liver without serious consequences. Eventually, the scarring can become so severe that the liver is no longer able to perform its normal functions.
Some of the most common causes of liver injury include:
People with cirrhosis may or may not have symptoms early in the course of the disease. Some of the more common symptoms include:
- Scarring makes it difficult for blood to flow through the liver. As a result, pressure backs up, and veins in other areas outside of the liver become abnormally expanded. Abnormally expanded blood vessels are referred to as varices.
- One place where varices are commonly found is in the esophagus, the swallowing tube connecting the mouth with the stomach (figure 2). When the pressure in the varices reaches a certain level, the varices can burst, which can cause massive bleeding (known as variceal bleeding or variceal hemorrhage).
- Body fluids accumulate as a result of liver scarring and a decreased ability to manufacture blood proteins. Fluid is typically seen in the legs (edema) and abdomen (ascites) and sometimes in the lung (pleural effusion). (See "Patient information: Edema (swelling) (Beyond the Basics)".)
- Ascites causes the abdomen to enlarge as fluid accumulates, which can cause shortness of breath and a feeling of fullness. The fluid provides an environment where bacteria can grow, increasing the risk of infection.
- Patients with cirrhosis can have easy bruising and bleeding. Once bleeding starts (such as with variceal bleeding), it can be severe.
- Hepatic encephalopathy is a condition that develops when the liver is unable to break down toxins normally found in the bloodstream, such as ammonia. In this condition, confusion or even coma are caused by toxins that build up in the blood. In the early stages, there may be mild symptoms, such as difficulty sleeping or sleeping too much. Advanced hepatic encephalopathy can cause confusion, delirium, and even coma. Hepatic encephalopathy can develop suddenly and may become a medical emergency.
- Patients with cirrhosis have a weakened immune system and are at increased risk of infections.
- Malnutrition is common in patients with cirrhosis. Malnutrition can cause loss of muscle in various areas of the body.
- Many people with advanced cirrhosis have jaundice (yellowed skin or whites of the eyes).
- People with cirrhosis are at increased risk for developing liver cancer (hepatocellular carcinoma).
- Cirrhosis can cause fatigue and in some cases itching.
Testing is performed to confirm the diagnosis of cirrhosis, determine the underlying cause, determine the severity of cirrhosis, and monitor for complications. (See "Cirrhosis in adults: Etiologies, clinical manifestations, and diagnosis".)
Liver biopsy — The best way to confirm the diagnosis of cirrhosis is a liver biopsy. This procedure is discussed in depth in a separate article. (See "Patient information: Liver biopsy (Beyond the Basics)".)
Imaging tests — Imaging tests, such as ultrasound, may be recommended to evaluate the condition of the liver or determine if there are cirrhosis-related complications. However, imaging tests are not usually performed to diagnose cirrhosis.
Blood tests — Blood tests may be performed to help determine the underlying cause of cirrhosis and to monitor the liver function over time.
There have been major advances in the treatment of cirrhosis in the past few decades. In particular, it has become easier to recognize, prevent, and treat complications of cirrhosis. (See "Cirrhosis in adults: Overview of complications, general management, and prognosis".)
People with cirrhosis should see their healthcare provider regularly for monitoring and treatment of cirrhosis complications. Although cirrhosis cannot be cured, several treatments are available to minimize cirrhosis-related complications. Other treatments are recommended to help prevent complications.
Avoid substances that can injure the liver — People with cirrhosis should avoid consuming substances that can further damage the liver. The most common of these is alcohol. You should talk to your healthcare provider before taking any new medication (including prescription and non-prescription drugs, herbs, vitamins, or dietary supplements).
Acetaminophen (Tylenol®) is a nonprescription medication that can further injure the liver in people with cirrhosis. The exact amount of acetaminophen that is safe in cirrhosis is uncertain; some experts recommend that patients not use more than 650 mg per dose every 4 to 6 hours, with no more than 2000 mg per day. However, even low doses may not be safe for those who drink alcohol. Use of acetaminophen should be discussed with a healthcare provider.
Nonsteroidal anti-inflammatory drugs such as ibuprofen (Advil®, Motrin®), naproxen (Aleve®, Naprosyn®), and aspirin can damage the liver and should be avoided if you have cirrhosis.
Screen for and treat varices — People with cirrhosis should undergo an upper endoscopy to determine if varices (expanded blood vessels) are present in the esophagus, the tube that connects the mouth and stomach (figure 2). Screening for and treatment of varices is discussed in detail in a separate topic review. (See "Patient information: Screening for esophageal varices (Beyond the Basics)" and "Prediction of variceal hemorrhage in patients with cirrhosis" and "Primary and pre-primary prophylaxis against variceal hemorrhage in patients with cirrhosis".)
Vaccination — People with cirrhosis should be vaccinated against hepatitis A and B. Pneumococcal vaccine and yearly influenza vaccine are also recommended. (See "Patient information: Adult vaccines (Beyond the Basics)" and "Patient information: Influenza prevention (Beyond the Basics)" and "Patient information: Pneumonia prevention (Beyond the Basics)".)
Treat ascites and edema — Ascites and edema can lead to complications, particularly infection. Ascites can also cause a person to feel short of breath or full. Thus, treatment is usually recommended to reduce the amount of fluid that collects in the lower legs and abdomen. Treatment usually involves taking one or more diuretic pills (fluid pills) and following a low-sodium (salt) diet. (See 'Dietary advice' below and "Ascites in adults with cirrhosis: Initial therapy".)
Paracentesis — Some people do not get adequate relief from edema and ascites with fluid pills alone. In this case, periodic drainage of the fluid (paracentesis) may be required. This is done by inserting a needle into the abdomen and withdrawing a large amount of fluid from the space around the abdominal organs. The procedure can usually be performed in a doctor's office.
Following paracentesis, it is important to continue taking your diuretic medication and to limit the amount of sodium you consume. (See 'Dietary advice' below.)
TIPS — A TIPS (transjugular intrahepatic portosystemic shunts) procedure may be recommended to treat ascites if diuretics, paracentesis, and changes in diet are not completely successful in relieving ascites. (See "Transjugular intrahepatic portosystemic shunts: Indications and contraindications".)
During the procedure, a radiologist places a device within the liver to reduce the blood pressure in the portal vein (in the liver) (figure 2). The procedure is usually performed with local anesthesia and sedation, and takes between one and three hours. Most patients remain in the hospital for one to three nights after the procedure.
Screen for hepatocellular carcinoma — People with cirrhosis should have tests to detect hepatocellular carcinoma (cancer of the liver). Testing usually requires an ultrasound examination of the liver every six months.
Consider liver transplantation — Not all patients with cirrhosis will require a liver transplantation, and many are not eligible for one. However, because the waiting list for liver transplantation is lengthy (up to two years in some regions), it is important to know if liver transplantation is a reasonable option while you are still relatively healthy. (See 'Liver transplantation for cirrhosis' below.)
Screen for encephalopathy — People with cirrhosis can develop confusion, which is sometimes subtle. Detecting confusion is important since treatment is available, and the confusion itself can lead to serious problems (eg, an automobile accident). A change in the sleep pattern (insomnia or sleeping too much) may be an early sign. Treatment may include a medication called lactulose (which will cause softening of the stool) or an antibiotic.
Dietary advice — People with advanced cirrhosis may require a specialized diet that includes lower amounts of salt. Salt restriction is usually recommended for people with early cirrhosis who tend to accumulate fluid. Protein restriction is usually not necessary. A healthcare provider or dietitian can help to determine if dietary changes are needed. A detailed discussion about a low-sodium diet is available separately. (See "Patient information: Low-sodium diet (Beyond the Basics)".)
The benefit of vitamins, antioxidants and other supplements on the underlying liver disease has not been established. Several herbal therapies have been reported as having a benefit in patients with cirrhosis. None have clearly been proven to be effective, although some continue to be studied. Most experts do not recommend vitamins, herbs, or other supplements for people with cirrhosis.
Exercise — Exercise is generally safe for people without advanced-stage cirrhosis. Exercise may increase the risk of variceal bleeding in patients with advanced disease (such as those who have ascites or varices). Thus, check with your healthcare provider regarding the risks and benefits of exercise.
LIVER TRANSPLANTATION FOR CIRRHOSIS
Liver transplantation involves replacing a diseased liver with a healthy liver. The majority of donated livers come from people who have suffered brain death for one reason or another. More recently, living donors have been able to donate a portion of their liver.
More than 80 percent of people will be alive one year after liver transplantation, and the majority of these will be alive five years after transplantation. This is compared with an extremely high death in patients with very advanced cirrhosis who do not undergo transplantation. (See "Model for End-stage Liver Disease (MELD)".)
The prognosis after transplantation depends in part upon the underlying cause of the liver disease, some of which recur following transplantation. As an example, most people who undergo transplantation for hepatitis C will develop recurrent hepatitis C after transplantation.
Other major concerns following transplantation are the risk of anti-rejection drugs used to suppress the immune system, which have many side effects, and the risk of rejection of the transplanted organ.
The transplantation process is elaborate, involving an extensive screening process for eligibility. Thus, not all patients with cirrhosis are eligible, and only those with the most advanced, severe cirrhosis are placed on the transplant registry. Furthermore, not all patients with cirrhosis will require a transplantation since the disease can remain relatively stable for many years, particularly if the cause of the cirrhosis (eg, alcohol) is eliminated. Treatment centers that perform liver transplantation can be found at the Centers for Medicare & Medicaid Services website (http://www.cms.gov/CertificationandComplianc/downloads/ApprovedTransplantPrograms.pdf). (See "Patient selection for liver transplantation".)
WHERE TO GET MORE INFORMATION
Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed on our Web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Patient information: Cirrhosis (The Basics)
Patient information: Swelling (The Basics)
Patient information: Hemochromatosis (The Basics)
Patient information: Hepatic encephalopathy (The Basics)
Patient information: Nonalcoholic steatohepatitis (NASH) (The Basics)
Patient information: Latest medicines for hepatitis C (The Basics)
Patient information: Liver cancer (The Basics)
Patient information: Fluid in the belly (ascites) (The Basics)
Patient information: Toxic hepatitis (The Basics)
Patient information: Esophageal varices (The Basics)
Patient information: Liver transplant (The Basics)
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
Patient information: Hepatitis B (Beyond the Basics)
Patient information: Hepatitis C (Beyond the Basics)
Patient information: Alcohol use — when is drinking a problem? (Beyond the Basics)
Patient information: Nonalcoholic steatohepatitis (NASH) (Beyond the Basics)
Patient information: Edema (swelling) (Beyond the Basics)
Patient information: Liver biopsy (Beyond the Basics)
Patient information: Screening for esophageal varices (Beyond the Basics)
Patient information: Adult vaccines (Beyond the Basics)
Patient information: Influenza prevention (Beyond the Basics)
Patient information: Low-sodium diet (Beyond the Basics)
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
Assessing surgical risk in patients with liver disease
Hepatic encephalopathy in adults: Clinical manifestations and diagnosis
Evaluation of adults with ascites
Cirrhosis in adults: Etiologies, clinical manifestations, and diagnosis
Epidemiology and etiologic associations of hepatocellular carcinoma
Ascites in adults with cirrhosis: Initial therapy
Management of pain in patients with advanced chronic liver disease or cirrhosis
Cirrhosis in adults: Overview of complications, general management, and prognosis
Prediction of variceal hemorrhage in patients with cirrhosis
Prevention of recurrent variceal hemorrhage in patients with cirrhosis
Primary and pre-primary prophylaxis against variceal hemorrhage in patients with cirrhosis
Ascites in adults with cirrhosis: Diuretic-resistant ascites
Transjugular intrahepatic portosystemic shunts: Indications and contraindications
Model for End-stage Liver Disease (MELD)
Patient selection for liver transplantation
The following organizations also provide reliable health information.
- National Library of Medicine
- National Institute of Diabetes and Digestive and Kidney Diseases
- American Gastroenterological Association
- The American Liver Foundation