WHAT IS IT?
Cirrhosis is a chronic liver disease characterized by the progressive and irreversible destruction of liver tissue as a result of viral infection, chronic exposure to alcohol, drugs, or toxic substance, or in association with another disease.
When exposed to an infectious agent, alcohol, or other toxic substance, liver cells may be killed faster than they can be replaced. Connective tissue cells then replace the liver cells, causing the liver to initially increase in size to compensate for the loss of function. If the scar tissue formation continues, however, the liver is unable to compensate adequately and may shrink in size. As the liver becomes smaller, blood flow may be forced to bypass the liver, causing a further decrease in function and the possibility of developing an enlarged spleen or other serious complication. In addition, if the liver is unable to function properly to remove, process, and break down metabolic products (such as protein), blood levels can reach toxic levels and damage other organs, including the brain.
There are numerous forms of cirrhosis that are distinguished by the known or suspected cause, the tissue changes observed in liver biopsies, and the symptoms that an individual exhibits. Alcohol-induced cirrhosis is the most common type, occurring in nearly fifteen percent of alcoholics. Cryptogenic or post-hepatitic cirrhosis occurs following some cases of infection with the hepatitis B or
hepatitis C virus. The cause of primary biliary cirrhosis, which involves the inflammation of tiny ducts that carry bile within the liver, is unknown. It tends to affect women between the ages of 35 and 60. Cirrhosis may also result from the accumulation of excessive amounts of either iron (hemochromatosis) or copper (Wilson's disease) within the liver or as a result of a congenital error of metabolism, as in alpha-1-antitrypsin enzyme deficiency.
At least 30,000 deaths per year (US) can be attributed to cirrhosis of the liver. Additionally, the liver cancer associated with some types of cirrhosis accounts for another 10,000 deaths annually.
HOW IS IT DIAGNOSED?
History: The different forms of cirrhosis share many clinical signs and symptoms. Individuals may report loss of appetite, abdominal pain, weight loss, yellowing of the skin (jaundice), water retention (edema,
ascites), and spider veins. Other signs and symptoms are unique to the type of cirrhosis.
Males with alcoholic cirrhosis may report an increase in breast size (gynecomastia) and a decrease in testicular size (testicular atrophy). Females may report menstrual irregularities or the complete cessation of menstruation (amenorrhea).
Physical exam: In primary biliary cirrhosis, the physical examination may be completely normal early on in the course of the disease. However, physical examination may reveal increased skin pigmentation, intense itching (
pruritus), dark urine, soft yellow spots of fat accumulation on the eyelids (xanthelasmas and xanthomas), an enlarged liver and spleen, and increased tissue at the end of the fingers (clubbing). In advanced cases, the findings resemble those of alcoholic cirrhosis.
Tests: In all cases, definitive diagnosis requires a liver biopsy in which a small piece of liver tissue is removed and examined microscopically for signs of inflammation, scarring, or infection. Other tests that may be performed include obtaining a complete blood count (CBC), measuring key liver enzymes, folate and vitamin B12 levels, serum chemistries, viral antibodies, serum immunoglobulin levels, and antibodies to DNA, smooth muscle, and mitochondria. Cholangiography (a procedure used to visualize the gallbladder duct and determine whether an obstruction is present) may be recommended for those individuals suspected of having primary biliary cirrhosis.
HOW IS IT TREATED?
Treatment is largely dependent upon the cause of the cirrhosis. Some methods of treating cirrhosis, regardless of the underlying cause, include dietary restrictions, such as placing an individual on a low-protein diet, supplementation with vitamins A, K, and D, and salt restrictions to reduce problems associated with fluid retention (ascites, edema). Individuals with alcoholic cirrhosis will be instructed to stop drinking alcohol. Individuals with primary biliary cirrhosis may be treated with antihistamines to relieve symptoms of itching and anti-inflammatory drugs, such as corticosteroids. In addition, liver transplants may be recommended for individuals with end-stage liver disease and ascites.
WHAT MIGHT COMPLICATE IT?
Almost all forms of cirrhosis are associated with portal hypertension, esophageal bleeding, enlarged spleen, fluid retention (ascites and edema), and coma. Other complications may include portal vein
thrombosis (blood clot formation), the development of liver tumors, altered drug metabolism, spontaneous bacterial peritonitis in which fluid accumulating within the abdomen becomes infected, and hepatic encephalopathy in which the brain is poisoned by high blood levels of certain metabolic byproducts.
PREDICTED OUTCOME
Although cirrhotic liver damage is permanent and irreversible, treatment is usually successful in prolonging life, decreasing morbidity, and preventing complications. Survival is a function of the severity of liver disease. Most individuals with cirrhosis will develop increasing jaundice, weakness, ascites, and portal hypertension within five years of diagnosis. Individuals with alcoholic cirrhosis who stop drinking have a five-year survival rate of 60% while those who continue to drink have a five-year survival rate of only 40%. Individuals with advanced alcoholic cirrhosis typically die sooner.Individuals with primary biliary cirrhosis die within five to ten years of diagnosis. After five years, approximately 67% are still alive. Individuals not exhibiting symptoms generally survive longer.
ALTERNATIVES
Differential diagnoses include other causes of liver cell injury, idiopathic portal hypertension, schistosomiasis, and congenital hepatic fibrosis.
APPROPRIATE SPECIALISTS
Gastroenterologist, pathologist, and general surgeon.