Primary biliary cirrhosis (PBC) is a disease of the liver in which the bile ducts in the liver slowly become destroyed. Bile is a fluid produced by the liver which aids in digestion and helps rid the body of worn-out red blood cells, cholesterol, and toxins.
When the bile ducts become destroyed, harmful substances start building up in the liver, contributing to irreversible scarring.
PBC is considered an autoimmune disease, as the body’s cells turn against its own tissues, causing the damage. As with many autoimmune diseases, the exact cause is unknown, but researchers believe it may be a combination of genetic and environmental factors.
As mentioned, because PBC is an autoimmune disease, the exact cause is unknown. How PBC can develop due to inflammation resulting from the accumulation of T cells within the liver as an immune response.
Normally, T cells recognize danger and work to defend the body, but in PBC, they wrongfully attack and destroy the cells of the biliary ducts within the liver.
Over time, inflammation starts spreading and healthy tissues get replaced with scar tissues, contributing to cirrhosis.
Risk factors for PBC include being female, being over the age of 30, genetic factors, infection, smoking, and environmental factors.
Complications that can arise as a result of PBC include cirrhosis, increased pressure in the portal veins, enlarged spleen, gallstones, enlarged veins, liver cancer, weak bones, vitamin deficiencies, decreased mental function, and an increased risk of other diseases such as metabolic syndrome and immune disorders like thyroid problems.
Symptoms of PBC in its early stages include fatigue, itchy skin, and dry eyes and mouth. As the disease progresses, symptoms include:
To properly diagnose PBC, your doctor will need to conduct several different types of tests, including blood tests to check liver function and blood tests to check for autoimmune disease. Other tests include ultrasound, CT scan, MRI, and X-ray of the bile ducts.
If after all these tests diagnosis is still uncertain, your doctor may perform a liver biopsy. A sample of the liver tissue will be removed and examined to check for disease.
There is no cure for PBC, but treatment options are available to reduce the risk of complications, help better manage the disease, and prolong life. Treatment options for PBC include:
Ursodeoxycholic acid (UDCA): This is a bile acid that helps move bile through the liver and may help prolong life if treatments starts in the early stages of the disease.
Liver transplant: If treatments are no longer successful and the liver begins to fail, a liver transplant may be required.
New medications: Researchers are working on new medications including new immunosuppressant drugs and antiviral medications.
Your doctor may also recommend treatments based on specific symptoms. For example, they may recommend antihistamines to treat itchiness, eye drops for dry eyes, and some treatments may be prescribed to reduce complications.
For example, you may need to up your vitamin intake to prevent deficiencies, take supplements to prevent bone loss, and other treatment to prevent portal hypertension, which is increased pressure in the portal vein.
Your doctor will choose the most suitable treatment option to control your condition, depending on your diagnosis and disease severity.