A chronic diffuse disorder of the liver, in which liver cells are destroyed and progressively replaced by scar tissue (fibrosis). The most common cause is long-continued excessive consumption of alcohol. Other causes include an auto-immune disorder, primary biliary cirrhosis, obstruction of the common bile duct (eg due to gallstones), and viral infections such as hepatitis B and C. It eventually leads to liver failure with jaundice, bleeding, accumulation of fluid in the peritoneal cavity within the abdomen, and eventually coma and death.
CirrhosisClassifications and external resources
| ICD-10 | K70, K71, K74 |
|---|---|
| ICD-9 | 571 |
Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. Cirrhosis is most commonly caused by alcoholism and hepatitis C, and was the 12th leading cause of death in the United States in 2000. Ascites is the most common complication of cirrhosis and is associated with a poor quality of life, increased risk of infections, and a poor long term outcome. In advanced stages of cirrhosis, the condition is irreversible and the only option would be a liver transplant.
Symptoms and signs
The following signs and symptoms may occur in the presence of cirrhosis or as a result of the complications of cirrhosis. Many are nonspecific and may occur in other diseases and does not necessarily point to cirrhosis. Likewise, the absence of any does not rule out the possibility of cirrhosis. Muehrcke's nails --- paired horizontal bands separated by normal color due to hypoalbuminemia Terry's nails --- proximal two thirds of the nail plate appears white with distal one-third red, also due to hypoalbuminemia Clubbing. This is due to increased estradiol and can occur up to 66% of patients. Due to congestion of the red pulp as a result of portal hypertension Ascites. Venous hum heard in epigastric region due to collateral connections between portal system and the remnant of the umbilical vein in portal hypertension. Sweet pungent smell in breath due to increased dimethylsulphide due to severe portal-systemic shunting. Yellow discoloring of the skin, eye, and mucus membranes due to increased bilirubin (at least 2-3 mg/dL). weakness, fatigue, anorexia, weight loss
Complications
As the disease progresses, complications may develop. Hepatic encephalopathy - the liver does not clear ammonia and related nitrogenous substances from the blood, which affect cerebral functioning: neglect of personal appearance, unresponsiveness, forgetfulness, trouble concentrating, or changes in sleep habits. Hepatocellular carcinoma is primary liver cancer, a frequent complication of cirrhosis. Portal hypertension - blood normally carried from the intestines and spleen through the portal vein flows more slowly and the pressure increases; Cirrhosis can cause immune system dysfunction, leading to infection. Cirrhosis can also lead to impotence, brain dysfunction and renal failure (hepatorenal syndrome) and osteoporosis.
Causes
Cirrhosis has many possible causes; In the Western World, chronic alcoholism and hepatitis C are the most common causes.
Alcoholic liver disease (ALD). Alcoholic cirrhosis develops in 15% of individuals who drink heavily for more than a decade. There is great variability in the amount of alcohol needed to cause cirrhosis (as little as 3-4 drinks a day in some men and 2-3 in some women). Alcohol seems to injure the liver by blocking the normal metabolism of protein, fats, and carbohydrates. 2.0, a value rarely seen in other liver diseases. Liver biopsy may show hepatocyte necrosis, Mallory bodies, neutrophilic infiltration with perivenular inflammation. Infection with this virus causes inflammation of and low grade damage to the liver that over several decades can lead to cirrhosis. The hepatitis B virus is probably the most common cause of cirrhosis worldwide, especially South-East Asia, but it is less common in the United States and the Western world. Hepatitis B causes liver inflammation and injury that over several decades can lead to cirrhosis. Hepatitis D is dependent on the presence of hepatitis B, but accelerates cirrhosis in co-infection. In NASH, fat builds up in the liver and eventually causes scar tissue. This disorder is similar to that of alcohol liver disease but patient does not have an alcohol history. Biopsy is needed for diagnosis and appears similar to that in alcohol liver disease. Gold standard diagnosis is antimitochondrial antibodies with liver biopsy as confirmation if showing florid bile duct lesions. This disease is caused by the immunologic damage to the liver causing inflammation and eventually scarring and cirrhosis. Cirrhosis due to autoimmune hepatitis still has 10-year survival of 90%+. Usually presents with family history of cirrhosis, skin hyperpigmentation, diabetes mellitus, pseudogout, and/or cardiomyopathy, all due to signs of iron overload. 71 umol/g, it is highly suggestive of homozygous HH and warrants genetic testing or liver biopsy. Heterozygotes do not develop cirrhosis. Autosomal recessive disorder characterized by low serum ceruloplasmin and increased hepatic copper content on liver biopsy. Due to chronic right sided heart failure which leads to liver congestion.Diagnosis
The gold standard for diagnosis of cirrhosis is a liver biopsy, through a percutaneous, transjugular, laparoscopic, or fine-needle approach. Histologically cirrhosis can be classified as micronodular, macronodular, or mixed, but this classification has been abandoned since it is nonspecific to the etiology, it may change as the disease progresses, and serological markers are much more specific. However, a biopsy is not necessary if the clinical, laboratory, and radiologic data suggests cirrhosis. However, normal aminotransferases do not preclude cirrhosis. Typically much higher in chronic liver disease from alcohol. Bilirubin -- May elevate as cirrhosis progresses. Albumin -- Levels fall as the synthetic function of the liver declines with worsening cirrhosis since albumin is exclusively synthesized in the liver Prothrombin time -- Increases since the liver synthesizes clotting factors. Globulins -- Increased due to shunting of bacterial antigens away from the liver to lymphoid tissue. Thrombocytopenia -- Due to both congestive splenomegaly as well as decreased thrombopoietin from the liver. Coagulation defects -- The liver produces most of the coagulation factors and thus coagulopathy correlates with worsening liver disease.
Imaging
Ultrasound is routinely used in the evaluation of cirrhosis, where it may show a small and nodular liver in advanced cirrhosis along with increased echogenicity with irregular appearing areas.
Other tests include abdominal CT and liver MRI, as well as nuclear studies.
Pathology
Macroscopically, the liver may be initially enlarged, but with progression of the disease, it becomes smaller. Depending on the size of the nodules there are three macroscopic types: micronodular, macronodular and mixed cirrhosis. In micronodular form (Laennec's cirrhosis or portal cirrhosis) regenerating nodules are under 3 mm. In macronodular cirrhosis (post-necrotic cirrhosis), the nodules are larger than 3 mm. The mixed cirrhosis consists in a variety of nodules with different sizes. Microscopically, cirrhosis is characterized by regeneration nodules, surrounded by fibrous septa. Fibrous septa are important and may present inflammatory infiltrate (lymphocytes, macrophages) If it is a secondary biliary cirrhosis, biliary ducts are damaged, proliferated or distended - bile stasis. 1
Pathophysiology
The liver plays a vital role in synthesis of proteins (e.g.
Cirrhosis is often preceded by hepatitis and fatty liver (steatosis), independent of the cause.
The pathological hallmark of cirrhosis is the development of scar tissue that replaces normal parenchyma, blocking the portal flow of blood through the organ and disturbing normal function. Iredale (2003) summarises the pivotal role of stellate cell, a cell type that normally stores vitamin A, in the development of cirrhosis.
The fibrous tissue bands (septa) separate hepatocyte nodules, which eventually replace the entire liver architecture, leading to decreased blood flow throughout. Portal hypertension is responsible for most severe complications of cirrhosis.
Treatment
Liver damage from cirrhosis cannot be reversed, but treatment can stop or delay further progression and reduce complications. A healthy diet is encouraged, as cirrhosis may be an energy-consuming process. Salt restriction is often necessary, as cirrhosis leads to accumulation of salt (sodium retention).
Treatment exists of elimination of the causes and preventing complications:
Elimination of causes: alcoholic cirrhosis caused by alcohol abuse is treated by abstaining from alcohol. Treatment for hepatitis-related cirrhosis involves medications used to treat the different types of hepatitis, such as interferon for viral hepatitis and corticosteroids for autoimmune hepatitis. Cirrhosis caused by Wilson's disease, in which copper builds up in organs, is treated with chelation therapy (e.g. For portal hypertension, propranolol is a commonly used agent to lower blood pressure over the portal system.In severe complications from portal hypertension, transjugular intrahepatic portosystemic shunting is occasionally indicated to relieve pressure on the portal vein.
If complications cannot be controlled or when the liver ceases functioning, a liver transplant is necessary. Survival from liver transplantation has been improving over the 1990s and is now around 90%, depending largely on the severity of disease in the recipient.
Coffee has recently (June 2006) been found to cut cirrhosis risk.
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