Cirrhosis is scarring of the liver caused by long-term inflammation or disease of the liver. Scar tissue replaces the normal healthy liver tissue, causing impairment of liver function and blockage of blood flow through the liver. As a result, the liver becomes leathery and nodular. A healthy liver makes proteins, helps fight infections, cleans the blood, helps digest food and stores a form of sugar that is used by the body for energy. A severely scarred, or cirrhotic, liver cannot function properly and can cause various symptoms and ill effects. One cannot live without a working liver but early treatment can control the symptoms and keep cirrhosis from getting worse.

WHAT CAUSES CIRRHOSIS?

  • Heavy alcohol use
  • Chronic hepatitis C (and less commonly B and D)
  • Nonalcoholic fatty liver disease, which is often caused by obesity
  • Auto-immune hepatitis, wherein the body’s immune system destroys the liver cells
  • Some drugs, medicines and harmful chemicals
  • Infections
  • Diseases that damage or destroy bile ducts (tubes that carry bile from the liver)
  • Some inherited diseases
  • Hemochromatosis: a disease that leads to iron deposition in the liver
  • Wilson’s disease: a condition that results in accumulation of copper in the liver

WHAT ARE THE SYMPTOMS OF CIRRHOSIS?
There usually are no symptoms in early stages of cirrhosis. As cirrhosis gets worse one may develop:

  • Weakness and tiredness
  • Loss of appetite
  • Abdominal discomfort
  • Weight loss
  • Spider shaped blood vessels under the skin

Cirrhosis may lead to more serious problems such as:

  • Easy bruising because of impaired production of clotting proteins by the cirrhotic liver and decreased platelets as a result of an enlarged spleen, which is often associated with liver cirrhosis
  • Bloating or swelling may occur due to fluid buildup in the abdomen (ascites) and legs (edema)
  • Waste materials may build up in the blood and brain as a result of loss of “refinery function” of the liver, resulting in confusion and sleepiness (hepatic encephalopathy)
  • Increased pressure in the veins entering the liver because the leathery cirrhotic liver does not allow normal free flow of blood through it (portal hypertension). This in turn leads to engorgement of collateral veins causing varices in the esophagus and stomach that can rupture and cause serious and life threatening bleeding resulting in vomiting of blood or blood in the bowel movement.
  • Yellow discoloration of the skin and eyes, called jaundice, due to increased deposition of bilirubin pigment
  • Serious itching when bile ducts are blocked
  • A small number of patients do develop liver cancer as a complication of liver cirrhosis.

HOW IS CIRRHOSIS DIAGNOSED?

By physical examination the doctor can identify the signs of cirrhosis, which include spider veins, jaundice, protuberant abdomen, leg swelling, a certain smell on the breath called fetor hepaticus (a musty sweet odor), flapping tremor of the extremities, bulging veins that snake out from the belly button (caput medusae), enlarged liver (in later stages the liver actually shrinks in size) and enlarged spleen.

Blood tests help doctors further evaluate for liver disease and determine the liver function. These may include CBC (complete blood count), blood chemistry tests, liver function tests (LFTs), test of clotting proteins such as prothrombin time (PT) and a whole host of specialized tests to zero in on various types of hepatitis and other chronic liver disorders. An elevated plasma ammonia level is associated with liver related confusion or stupor (hepatic encephalopathy) and often is measured as well. Blood tests in conjunction with examination findings allow the doctors to grade the severity of cirrhosis.

Imaging tests, such as an abdominal sonogram, a CT and an MRI, are performed to evaluate the size, shape and texture of the liver, size of the spleen and to exclude tumors.

Occasionally, a liver biopsy is done but, in most cases, the physical examination, blood tests and imaging studies are enough to make the diagnosis of cirrhosis.

HOW IS CIRRHOSIS TREATED?

There is no treatment that can make the scar tissue of cirrhosis go away. But, luckily, the liver does regenerate, or produce new cells, and by removing the cause or treating it, cirrhosis can be kept from getting worse, e.g., complete cessation of drinking alcohol in alcoholic liver cirrhosis, treatment of hepatitis C or auto-immune hepatitis with medicine. Treatment generally is based on the cause of the cirrhosis and symptoms the patient has. Patients with cirrhosis need to exercise caution in taking medications, including over the counter vitamins and herbal supplements, because of their ill effects on the liver as well as altered metabolism of the drugs by the cirrhotic liver. Patients with cirrhosis need to be vaccinated against hepatitis A and hepatitis B. Even though hepatitis A does not cause cirrhosis, it can damage the liver further in a cirrhosis patient. A flu shot and vaccination against pneumonia are also recommended. Avoidance of eating raw oysters and other raw shellfish is important as these can harbor bacteria that can cause serious infections in people with cirrhosis.

TREATMENT OF COMPLICATIONS EDEMA AND ASCITES

These conditions are treated by a low-salt diet and water pills (diuretics) like spironolactone and furosemide. If the ascites persists, one might need removal of the fluid via insertion of a small needle into the abdominal cavity in a procedure called paracentesis. Occasionally, the fluid may become infected with bacteria, a condition called spontaneous bacterial peritonitis (SBP), which can be serious, and requires antibiotic therapy.

BLEEDING FROM VARICES

Because of portal hypertension, there is engorgement of veins in the esophagus and upper stomach, resulting in varicose veins called varices, which can rupture and bleed. The bleeding is usually severe and can be fatal without prompt treatment. The variceal bleeding usually causes the patient to vomit blood or coffee ground-looking material and/or have dark stools (called melena: blood made dark by exposure to stomach acid). These patients need urgent hospitalization and require blood transfusions and medications to lower the pressure of abdominal veins. The bleeding varices are typically treated with endoscopy and the bleeding veins are tied off using rubber bands or are injected with sclerosing agents to stop the blood flow and to obliterate the varices. If these measures fail, a shunt can be deployed in the liver connecting the portal vein and hepatic vein, thus decompressing the high pressure system. This is called TIPS, or transjugular intrahepatic portosystemic shunt, and is placed via the neck veins by specially trained radiologists. While TIPS is very effective in controlling the bleeding, it can result in elevated ammonia levels and encephalopathy because the blood flow through the shunt actually bypasses whatever little functional liver tissue that remains in the cirrhotic liver.

HEPATIC ENCEPHALOPATHY

This condition results from the declined refining capacity of the liver or decreased ability to remove the toxins from the food before it enters general circulation. These toxins essentially short circuit the brain and cause mental confusion, increased or altered sleep pattern and sometimes frank coma. The treatment involves restricting the patient to a 70g-protein diet and giving medications like lactulose and/or Rifaximin.

LIVER FAILURE

If the treatment measures outlined above do not work and the cirrhosis patient develops relentless worsening of the liver function resulting in liver failure, liver transplant can be a life saving option.

By S Murthy Badiga, MD, FACG