Cirrhosis is a condition in which a chronically diseased liver gradually develops hard scar tissue (fibrosis) and grows lumps (regenerative nodules) in an attempt to repair itself. Eventually, cirrhosis progresses throughout the liver resulting in irreversible liver damage and impaired liver function. The clinical features of liver failure develop because blood flow in the liver becomes obstructed and the liver loses its normal ability to support digestion, metabolize toxins, and produce proteins for normal clotting function.
Causes and Risk Factors
Any progressive liver disease may lead to cirrhosis. Alcoholism is by far the most common cause of cirrhosis, followed by chronic hepatitis C, nonalcoholic fatty liver disease, and chronic hepatitis B.
Less common etiologies include bile duct diseases (such as biliary atresia, primary sclerosing cholangitis, and primary biliary cirrhosis), cystic fibrosis, autoimmune hepatitis, and inherited metabolic liver diseases (such as hemochromatosis, Wilson’s disease, and alpha-1 antitrypsin deficiency). Also, drug toxicity and heart failure may cause cirrhosis.
Cirrhosis progresses through two stages:
- An initial stage with no symptoms, called compensated cirrhosis
- A late stage called decompensated cirrhosis, characterized by severe symptoms and complications
For many years, a patient may remain in the compensated stage without symptoms until finally decompensation occurs.
The symptoms of decompensation include anorexia, fatigue, nausea, vomiting, abdominal pain, and itching. All symptoms and complications of cirrhosis are the result of two main consequences of the disease: portal hypertension and liver insufficiency.
Portal hypertension occurs as blood flow in the liver becomes obstructed and pressure increases in the portal vein. This condition leads to accumulation of fluid in the abdominal cavity (ascites), pleural effusions, edema of the legs, dilation of superficial veins of the abdomen, and dilation of veins in the esophagus (varices). Upper gastrointestinal tract bleeding is a potentially life-threatening complication of esophageal varices.
Ascites may disrupt kidney function (hepatorenal syndrome), and ascitic fluid can become infected resulting in spontaneous bacterial peritonitis.
An enlarging spleen (hypersplenism) sequesters platelets, thus lowering the platelet count (thrombocytopenia) and interfering with clotting function. Furthermore, a failing liver produces inadequate clotting factors and increases the risk for bleeding.
In late-stage cirrhosis, the liver becomes unable to excrete bilirubin (a normal waste product of red blood cells), which causes yellowing of the skin (jaundice) and eyes (sclera icterus) as bilirubin accumulates in the body.
A normal liver is capable of removing ammonia and other toxins from the body. However, as liver function declines, these toxins accumulate in the brain resulting in hepatic encephalopathy characterized by confusion, delirium, and ultimately coma.
Chronic liver diseases, such as cirrhosis, are also associated with hepatocellular carcinoma, the most common kind of liver cancer, which carries a very high mortality rate.
Diagnosis and Treatment
The physical examination of a patient with cirrhosis may reveal abdominal swelling, muscle atrophy, spider angiomas, palmar erythema, and pinpoint bleeding in the skin (petechiae). The liver may feel lumpy (nodular) and an enlarged spleen may be present. Males can have testicular atrophy and excessive breast tissue (gynecomastia), while women may experience an absence of menstruation (amenorrhea).
Laboratory tests can be unremarkable or show an elevated bilirubin level, low levels of a protein called albumin, thrombocytopenia, and impaired clotting as evidenced by an elevated prothrombin time. Further testing with ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) detects the presence of a cirrhotic liver; however, a liver biopsy remains the diagnostic gold standard.
In cirrhosis, the areas of scar tissue are permanently damaged and cannot be reversed, so treatment is aimed at correcting the underlying liver disease and avoiding liver toxins to prevent further damage. Alcohol should be avoided, and if hepatitis is present, an antiviral medication may be prescribed.
Ascites is initially controlled with diuretic medications, which increase urination, such as spironolactone and furosemide. When diuretic medications fail, ascitic fluid can be directly withdrawn through a needle inserted in the peritoneal cavity (paracentesis).
Esophageal varices may require a procedure called endoscopic variceal ligation in which rubber rings are positioned to prevent variceal bleeding. Actively bleeding varices are treated with blood transfusion, an endoscopic procedure, and an intravenous medication (octreotide) to decrease the variceal blood flow. Patients with repeated episodes of variceal bleeding may require surgery or insertion of a transjugular intrahepatic portosystemic shunt (TIPS).
Hepatic encephalopathy is often managed with lactulose, a daily medication to reduce the body’s production of ammonia. Additionally, an antibiotic may be used to decrease the level of ammonia-producing bacteria in the intestines.
Liver transplantation, replacement of the diseased liver with a healthy organ from a donor, is an option when complications of the disease remain uncontrolled and the risk of mortality from the disease exceeds the risk of the surgery. Currently, the shortage of organs is the main limiting factor.
Given the irreversible nature of cirrhosis and difficult treatment, great emphasis is placed on preventing common liver diseases or halting the progression of any liver disease as early as possible.
Individuals can decrease their risk for acquiring a liver disease by limiting alcohol consumption and avoiding behaviors that increase the risk for hepatitis, such as unprotected sex and intravenous drug use. The most beneficial intervention is early treatment of any underlying liver disease before progression to cirrhosis.
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