PRIMARY BILIARY CIRRHOSIS - Overview, Facts, Types, Symptoms, etc
Primary Billiary

PRIMARY BILIARY CIRRHOSIS

Primary biliary cirrhosis is a progressive disease of the liver that results from the accumulation of bile within the liver (cholestasis). Damage to the small bile ducts that drain bile from the liver usually follows. Liver cell damage usually results from the pressure build-up that destroys the bile ducts. Cirrhosis and liver failure occur when a considerable amount of liver cells die. This happens when the disease is in its advanced stages.

Bile is a substance that is produced in the liver that is then transported through the bile ducts and to the gallbladder and intestine where it aids in the digestion of lipids and lipid-soluble vitamins (A, D, E, K). Inflammation and tissue necrosis occurs when bile cannot be drained out of the liver. Scar tissue and fat gradually replace the damaged liver tissues; the liver could therefore not perform its usual functions.

SYMPTOMS

Symptoms of primary biliary cirrhosis may include the following:

  • Easy bruising from decreased production of blood clotting factors by the damagedliver
  • Fatigue
  • Itching
  • Loss of appetite
  • Weakness
  • Yellowing of the skinand eyes (jaundice) due to the accumulation of bilirubin in the blood

DIAGNOSIS

Diagnosis of primary biliary cirrhosis is done when the physician suspects that there might be liver inflammation upon checking your medical history and performing a physical examination.

 

When abnormal liver functions are found on routine blood tests drawn for other reasons, a diagnosis may be made for primary biliary cirrhosis. Liver function tests include ALT, AST, ALP, bilirubin, and GGT. The blood levels of these enzyme markers may be elevated in specific patterns depending upon the underlying cause of inflammation when a patient is suffering from liver disease.

To confirm the suspected diagnosis, a blood test to check for antimitochondrial antibody (AMA) is usually done. Over 90% of patients with primary biliary cirrhosis flag as positive in this test.

A complete blood count (CBC) and lipid profile may be also considered in making a diagnosis.

To look for structural changes in the liver and to search for tumors, imaging of the abdomen through ultrasound, CT scan, or MRI is often considered.

To further help confirm the diagnosis, a liver biopsy – a process wherein a thin needle is inserted through the skin to take a sample of tissue. It is examined by a pathologist to look for changes that are characteristic of primary biliary cirrhosis.

TREATMENT

Liver transplant is the only cure for primary biliary cirrhosis, however, it is only considered as the “treatment of last resort” if other less invasive treatment options have failed and the patient succumbs to liver failure.

The first-line treatment for primary biliary cirrhosis is ursodiol or ursodeoxycholic acid. In an attempt to prevent cholestasis, it aids in the transport of bile into the gallbladder and intestine.

Immunosuppressants may be prescribed depending on the severity of the disease. These include methotrexate, cyclosporine, and prednisone.

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