Patient information: Cirrhosis

CIRRHOSIS OVERVIEW

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 of cirrhosis include longstanding alcohol abuse and hepatitis B or hepatitis C. 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" and "Patient information: Hepatitis C" and "Patient information: Alcohol use — when is drinking a problem?".)

CIRRHOSIS CAUSES

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:

• Longstanding alcohol abuse (see "Patient information: Alcohol use — when is drinking a problem?".
• Chronic hepatitis (B or C) (see "Patient information: Hepatitis B" and "Patient information: Hepatitis C".
• Nonalcoholic steatohepatitis, a condition that causes inflammation and accumulation of fat and fibrous tissue in the liver. (See "Patient information: Nonalcoholic steatohepatitis (NASH)".)

CIRRHOSIS SYMPTOMS

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, 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).

• 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)".)

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 are at risk of 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 DIAGNOSIS

Testing is performed to confirm the diagnosis of cirrhosis, determine the underlying cause, determine the severity of cirrhosis, and monitor for complications. (See "Diagnostic approach to the patient with cirrhosis".)

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".)

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.

CIRRHOSIS TREATMENT

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 "Overview of the complications, prognosis, and management of cirrhosis".)

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 injury 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.

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" and "Prediction of variceal hemorrhage in patients with cirrhosis" and "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 immunizations" and "Patient information: Influenza prevention", and see Patient information: Pneumonia prevention").

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 "Initial therapy of ascites in patients with cirrhosis".

Paracentesis — Some people do not get adequate relief of 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 "Indications and contraindications to the use of transjugular intrahepatic portosystemic shunts".)

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 and a blood test every 6 to 12 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.

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. 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".)

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.

Approximately 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 to almost certain 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 are listed in table 1 (table 1). (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. Because no two people are exactly alike and recommendations can vary from one person to another, it is important to seek guidance from a provider who is familiar with your individual situation.

This discussion will be updated as needed every four months on our web site (www.uptodate.com/patients). Additional topics as well as selected discussions written for healthcare professionals are also available for those who would like more detailed information.

Some of the most pertinent include:

Patient Level Information:
Patient information: Hepatitis B
Patient information: Hepatitis C
Patient information: Alcohol use — when is drinking a problem?
Patient information: Nonalcoholic steatohepatitis (NASH)
Patient information: Edema (swelling)
Patient information: Liver biopsy
Patient information: Screening for esophageal varices
Patient information: Adult immunizations
Patient information: Influenza prevention
Patient information: Low sodium diet

Professional Level Information:
Assessing surgical risk in patients with liver disease
Clinical manifestations and diagnosis of hepatic encephalopathy
Diagnosis and evaluation of patients with ascites
Diagnostic approach to the patient with cirrhosis
Epidemiology and etiologic associations of hepatocellular carcinoma
Initial therapy of ascites in patients with cirrhosis
Management of pain in patients with cirrhosis
Overview of the complications, prognosis, and management of cirrhosis
Prediction of variceal hemorrhage in patients with cirrhosis
Prevention of recurrent variceal hemorrhage in patients with cirrhosis
Primary prophylaxis against variceal hemorrhage in patients with cirrhosis
Treatment of diuretic-resistant ascites in patients with cirrhosis
Indications and contraindications to the use of transjugular intrahepatic portosystemic shunts
Model for End-stage Liver Disease (MELD)
Patient selection for liver transplantation

A number of web sites have information about medical problems and treatments, although it can be difficult to know which sites are reputable. Information provided by the National Institutes of Health, national medical societies and some other well-established organizations are often reliable sources of information, although the frequency with which they are updated is variable.

• National Library of Medicine

      (www.nlm.nih.gov/medlineplus/healthtopics.html)

• National Institute of Diabetes and Digestive and Kidney Diseases

      (www.niddk.nih.gov/)

• American Gastroenterological Association

      (www.gastro.org/wmspage.cfm?parm1=681)

• The American Liver Foundation

      (www.liverfoundation.org)

[1-4]