Cirrhosis

What is Cirrhosis?

Cirrhosis is a disorder of the structure of the liver that occurs when there is continuing liver damage over a prolonged period of time, usually a few years or more. Although alcoholism usually comes to mind when cirrhosis is mentioned, there are many other causes of this disorder.

Obviously, alcoholic damage is a frequent cause of cirrhosis all over the world but there are other causes of liver injury including prolonged hepatitis virus infection, autoimmune liver disease (when the body's immune system causes damage to the liver), excess iron in the liver, and adverse reactions to medications. Even obesity, in some cases, is a well recognized cause of cirrhosis.

Illustration of a normal liver next to a liver with cirrhosis.When there is continuing liver injury the liver attempts to repair the damage by replacing lost liver tissue (a process called regeneration) and by forming scars. Unfortunately this often leads to the formation of irregular nodules within scar tissue in the liver. If the cause of liver injury is removed, progression of the cirrhosis may stop although the scarring usually remains. The cause of liver injury may be removed, for example, by abstaining from alcohol, by successfully treating viral infection, by removing excess iron from the body, by avoiding harmful medications or by treating autoimmune inflammation with drugs.

What Are The Consequences of Cirrhosis?

There are three main consequences associated with the presence of cirrhosis.

(i) The most common and frequent cause of ill health in patients with cirrhosis results from interference with normal blood flow into the liver, caused by the abnormal structure of the liver. The liver is unusual because it receives blood from two sources. Not unlike many other organs, the liver receives oxygen-enriched blood from the heart via an artery. The liver also receives blood that is rich in nutrients from various parts of the gastrointestinal tract via a special vein called the portal vein. When the liver is scarred and distorted the flow of blood into the liver through the portal vein is impaired. This leads to a rise in pressure in the portal vein (which doctors call portal hypertension). The blood is forced to bypass the liver and return to the general circulation through veins that do not normally carry a large blood flow.

Endoscopic view of varicose veins in the esophagus. 
Varicose veins in the esophagus.

These veins are present in the stomach and esophagus, as well as other places. These veins become enlarged and irregular and are called varicose veins. However, unlike the varicose veins that are seen on the legs (which are covered by skin), these varicose veins are covered only by the delicate lining of the throat and stomach and easily bleed. Reduced blood flow into the liver also causes congestion and enlargement of the spleen which in turn leads to a reduction in blood count (usually affecting the platelets and white cells of the blood). Another consequence of the abnormal liver structure is the tendency for the liver to weep fluid from its surface into the abdominal cavity, causing distention. There is frequently fluid retention in the abdomen (which doctors call ascites). There may be fluid retention in the legs as well, as a result of poor liver function. A higher-level complication that can accompany fluid retention and portal hypertension is reduced function of the kidney. This usually occurs only in the most advanced stages of cirrhosis.

(ii) The second consequence of cirrhosis is an overall impairment of the function of the liver, because of the loss of liver tissue and the abnormality of blood flow mentioned above. Major functions of the liver include the manufacture of proteins and clotting factors for the blood, getting rid of waste substances from the body, and dealing with and removal of foreign substances such as medications. When the liver functions poorly, blood proteins may be reduced (which leads to fluid retention in the legs) and clotting may be poor. When the blood count (especially the platelet count) is reduced and clotting factors are deficient, there is an increased risk of bleeding with injury, surgery, dental extractions, etc. The reduced ability of the liver to get rid of waste substances may lead to jaundice (yellowing of the eyes and skin), drowsiness, confusion and even unconsciousness. Reduction in the body's ability to get rid of medications may lead to excessive medication effects, such as drowsiness with sedatives and sleeping tablets.

(iii) The third consequence of cirrhosis is not related to the function of the liver. Rather, there is an increased risk of liver cancer after cirrhosis has been present for many years. This may occur in any form of cirrhosis, but cancer is more common when cirrhosis is due to hepatitis virus infection, alcohol, increased iron in the liver and several other causes.

How Can Cirrhosis Be Treated?

Obviously the best treatment for cirrhosis is its prevention in the first place. Certain behaviors that can potentially lead to hepatitis virus infection (ex. needle sharing, unprotected sex, and excessive alcohol intake) should be avoided. Hepatitis viruses are readily spread by needle sharing and, in the case of hepatitis B infection, by unprotected sexual intercourse with a partner who is a carrier of the virus. Hepatitis viruses were spread by blood transfusion once. This is now very unlikely indeed as the blood supply is so well tested. Once liver injury is present, it is important to remove its cause, or use specific treatment to counteract it. These treatments will differ according to the cause of the liver disease.

How Are Complications of Cirrhosis Treated?

If a patient has already bled from varicose veins in the esophagus or stomach, the veins that are present can be treated by direct injection or by rubber banding via endoscopy. The pressure in these varicose veins may also be reduced by using certain medications (such as Inderal or Corgard). In some situations, a radiologist (X-ray doctor) can place a special tube through the liver connecting the inflow vein to the outflow vein, so that blood does not flow into the varicose veins. This is called a Transhepatic Intrajugular Portosystemic Shunt (TIPS).

Fluid retention is best treated by combination of a low salt diet and the use of fluid tablets (called diuretics), which increase urine production and fluid loss from the body. The common fluid tablets are spironolactone (Aldactone) and furosemide (Lasix) but there are many others. Occasionally excessive fluid in the abdomen must be removed by direct drainage with a needle and tube. In extreme cases, a TIPS tube placed in the liver can also help with fluid retention. TIPS is more commonly used to treat bleeding varicose veins in the esophagus or stomach than fluid retention.

What Are Other Considerations In Cirrhosis?

If confusion or drowsiness is a problem, a syrup called lactulose is used in mild laxative doses that lead to a slight increase in bowel movements (no more than 3-4 daily). This often improves thinking and consciousness.

Patients with cirrhosis who have a bleeding tendency, may need to be given a transfusion of clotting factors, prior to or during such procedures as dental extraction and surgery. Frequently, doses of a variety of medications (such as sedatives, pain killers, or even antibiotics) must be reduced for use in patients with cirrhosis.

When cirrhosis is advanced and liver function is poor, and when there is difficulty controlling bleeding from varices, liver transplantation may be the appropriate treatment.

What Is The Outlook For Someone With Cirrhosis?

It should be emphasized, that in many cases, neither the patient nor the physician are aware of the fact that cirrhosis is present. Patients may have cirrhosis for many years without any reduction in health. However once complications of cirrhosis occur, this is usually accompanied by some reduction in life expectancy, depending on the cause and severity of the decrease in liver function. It is important to remember that treating the cause of cirrhosis is almost always worthwhile, as it is likely to lead to an improvement in liver function, health and improvement in patient's life expectancy.