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HomeHealth articlesprimary biliary cholangitisWhat Are the Causes of Primary Biliary Cholangitis?

Primary Biliary Cholangitis - Causes, Symptoms, Diagnosis, and Treatment

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Primary biliary cholangitis is a autoimmune chronic disorder destroying small ducts inside the liver. The article explains the condition.

Medically reviewed by

Dr. Jagdish Singh

Published At November 8, 2022
Reviewed AtJune 8, 2023


Primary biliary cholangitis is a chronic disorder in which small bile ducts inside the liver become inflamed and eventually destroyed. This bile duct dysfunction leads to bile accumulation in the liver, thus causing liver damage. This liver damage over the time leads to scarring, cirrhosis, and liver failure. Primary biliary cholangitis is an autoimmune disorder which means the body's immune system becomes overactive and attacks the bile duct cells. The condition can also be called primary biliary cirrhosis. It is more common in women and affects middle-aged individuals.

What Are the Causes of Primary Biliary Cholangitis?

The causes of primary biliary cholangitis are unknown. however, some studies suggest that few genes help to develop the disease. These genes get triggered by some environmental factors and cause an autoimmune reaction that causes the body's immune system to attack normal small bile ducts in the liver and cause primary biliary cholangitis.

Some of the triggering environmental factors are:

What Are the Symptoms of Primary Biliary Cholangitis?

1) The basic and most common symptoms of primary biliary cholangitis are:

  • Feeling tiresome.

  • Itchy skin.

2) Other symptoms of primary biliary cholangitis are:

  • Joint pain or arthritis.

  • Dry legs and dry mouth due to conditions such as Sjogren’s syndrome.

  • Pain and discomfort in the upper right region of the abdomen.

  • Darkening of skin color due to pigmentation.

  • Symptoms of cirrhosis such as edema, jaundice, and weight loss.

  • Xanthomas formation. These are fatty deposits that appear as yellow bumps on the skin.

What Are the Complications Caused by Primary Biliary Cholangitis?

1) Some common complications of primary biliary cholangitis are:

  • Increased blood cholesterol levels.

  • Low levels of fat-soluble vitamins, which are A,D,E and K.

  • Loss of calcium from the bones leads to osteoporosis. Osteoporosis is a disorder characterized by weakening and decreasing bone density. The bones become fragile and fracture easily.

2) Liver Complications: Primary biliary cholangitis eventually damages the liver due to bile juice accumulation. The complications due to liver damage are:

  • Portal Hypertension: This condition occurs when the scar tissue in the liver, which suffered cirrhosis blocks the normal blood flow. This leads to increased blood pressure in the portal vein. When portal hypertension reaches a certain level, it can cause some additional complications, such as:

  • Ascites: Accumulation of fluid in the abdomen.

  • Edema: Swelling in legs, feet, or ankles.

  • Varices: Enlargement of veins in the stomach, esophagus, or intestine which can also cause internal bleeding due to the bursting of vein.

  • Hepatic Encephalopathy: It leads to confusion and difficulty in thinking as toxins accumulate in the brain.

  • Cirrhosis: The scar tissue replaces the healthy liver tissues and causes liver dysfunction. The end stage of cirrhosis is liver failure.

  • Liver Cancer: Studies have suggested that people with cirrhosis and liver failure due to primary biliary cholangitis have an increased risk of liver cancer.

How to Diagnose Primary Biliary Cholangitis?

1) The first step in diagnosing primary biliary cholangitis is medical and family history. It is important to take history of any autoimmune disorder in the patient or any one close in the family.

2) The second most important step in diagnosing primary biliary cholangitis is physical examination. It includes:

  • Palpation of specific regions of the stomach.

  • Checking for yellowish discoloration of the sclera of eyes or skin.

  • Checking the size of the liver and spleen by palpation.

  • Evaluation of abdominal tenderness or pain, especially in the upper right side of the abdomen.

3) Blood Tests: The blood test advised for primary biliary cholangitis is anti-mitochondrial antibodies (AMA). The presence of anti-mitochondrial antibodies is seen in 95% of people with primary biliary cholangitis.

4) Liver Tests: The liver test depicts abnormal liver enzyme levels, which helps to predict liver damage. Higher levels of enzyme alkaline phosphatase are seen in the case of primary biliary cholangitis.

5) Cholesterol Level Check: Cases of primary biliary cholangitis may have higher than normal cholesterol levels.

6) Imaging Tests: X-rays and ultrasounds rule out other disorders such as gallstones, bile duct strictures, or tumors that may show similar complications as primary biliary cholangitis.

7) Liver Biopsy: A small tissue sample from the liver is sent to check for any cancer or malignancies, as they may show similar symptoms of liver damage as primary biliary cholangitis.

What Are the Treatment Options for Primary Biliary Cholangitis?

The treatment of primary biliary cholangitis depends upon the complications caused by it.

1) The doctor may prescribe medicines to stop or slow bone loss for osteoporosis. Dietary supplements such as calcium and vitamin D are also suggested.

2) For lower levels of fat-soluble vitamins A.D.E and K, dietary supplements are advised.

3) If the patient has abnormal cholesterol levels, medicines such as statins are prescribed along with dietary changes.

4) Regular eye and dental examinations are advised in cases of dry eyes and mouths.

5) The liver complications are treated depending on severity:

  • Cirrhosis or Portal Hypertension: These conditions are treated with medicines, minor medical procedures, and surgery.

  • Liver Failure: In cases of liver failure, a transplant is the only choice of treatment.

  • Liver Cancer: Diagnosis of cancer at an early stage helps to cure the disease.


Primary biliary cholangitis is an autoimmune disorder triggered by environmental factors such as infections, cigarette smoking, or exposure to certain chemicals. The condition is characterized by the destruction of small bile ducts inside the liver, leading to the accumulation of bile in the liver. This building up of bile in the liver can cause tissue scarring, cirrhosis, and eventually liver failure. The treatment depends on the complication caused by the disease.

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Dr. Jagdish Singh
Dr. Jagdish Singh

Medical Gastroenterology


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