Primary biliary cirrhosis is a chronic, progressive condition affecting the bile ducts of the liver. The irritation and swelling of these ducts is accompanied by blockage of bile flow. As a result, the obstruction leads to damage to liver cells which eventually causes liver cirrhosis.
Primary Biliary Cirrhosis Causes and Risk Factors
It has not been fully understood why bile ducts get inflamed in the first place. What is estimated is that primary biliary cirrhosis in many cases affects middle-aged women. Obstruction that lasts for a long period of time is responsible for severe damage to the liver and the onset of liver cirrhosis.
This medical condition may be closely related to some autoimmune disorders like celiac disease, Sicca syndrome, hypothyroidism or Raynaud's phenomenon. So people suffering from these conditions are actually at higher risk of developing primary biliary cirrhosis as well.
Primary Biliary Cirrhosis Clinical Characteristics
The condition is asymptomatic in around half of all patients at the time of diagnosis. Once the symptoms occur, they gradually intensify.
Abdominal pain is one of common complaints. Furthermore, there are liver enlargement, fatigue, fatty deposits under the skin, fatty stool, itching, jaundice and soft yellow spots on the eyelids.
Diagnosing Primary Biliary Cirrhosis
After performing physical examination and thorough investigating patient's medical history doctors opt for several more tests. They perform tests for liver dysfunction including serum albumin, liver function tests, prothrombin time, serum cholesterol and lipoproteins etc.
More specific tests which can confirm the condition are liver biopsy, conformation of elevated immunoglobulin M and the presence of anti-mitochondrial antibodies.Primary Biliary Cirrhosis Treatment
The goal of the treatment for primary biliary cirrhosis is to reduce the intensity of symptoms and prevent potential complications.
Cholestyramine is a drug that efficiently deals with itching. Removal of bile is accelerated by ursodeoxycholic acid. By eliminating bile, it cannot accumulate inside the bile ducts and cause inflammation. So this drug actually improves the survival of patients suffering from primary biliary cirrhosis.
Such patients additionally need vitamin replacement therapy (vitamins A, D and K), calcium supplements and only if cirrhosis is severe and is associated with advanced liver failure, one needs to undergo liver transplantation.
Primary Biliary Cirrhosis Prognosis and Potential Complications
Patients who are not treated inevitably end up with liver failure. Still, this complication may also affect patients who have been properly treated. It is estimated that around 25% of all patients who have been suffering from primary biliary cirrhosis for 10 years are going to develop liver failure.
The disease is associated with certain complications which generally develop when the disease reaches advanced stages. They include progressive cirrhosis accompanied by liver failure, bleeding, damage to the brain in a form of encephalopathy, fluid/electrolyte imbalance, malapsorption/malnutrition, kidney failure and osteomalacia.