Primary biliary cirrhosis, formerly known as primary biliary cholangitis, is an autoimmune disease of the liver that results from progressive damage to the small bile ducts. This causes bile toxins and many other toxins to build up in the liver.

But what are these bile ducts, and what is their importance? The bile ducts are channels located in the liver. They produce a substance called bile. This substance contributes to the digestive process and helps in the absorption of some types of vitamins. Also, it rids the body of cholesterol, toxins, and damaged red blood cells. From these functions, one can guess the serious complications that will affect the body as a result of the inflamed bile ducts.

Primary cholangitis begins when certain types of white blood cells called T- cells begin to collect in the liver. These normal immune cells are important for killing bacteria and viruses. But in the case of primary cholangitis, they destroy the healthy cells that line the small bile ducts in the liver.

The daily increased damage to the bile ducts leads to scarring or fibrosis of the entire liver.

Symptoms of primary biliary cholangitis

The patient does not suffer from any symptoms at the beginning of his infection with primary biliary cirrhosis. The symptoms appear about 5-10 years after infection. If the person is lucky, the disease may be discovered by chance during the diagnosis of another health problem of the body.

The most well-known symptoms of biliary tract infection are:

The picture shows different health
conditions of a liver
  1. Dry eyes and mouth.
  2. Pain in the upper right part of the abdomen.
  3. Bone pain.
  4. Muscle and joint pain.
  5. Enlargement of the spleen; one can detect this by lying on the back and feeling the swollen place of the spleen.
  6. Swollen feet and ankles
  7. Fatigue without a reason.
  8. Itchy skin all over the body.
  9. Flatulence: it is the cause of fluid buildup in the abdomen as a result of liver failure.
  10. Jaundice: it manifested in yellow skin, white eyes, white soles of the feet, and white soles of the hands.
  11. Skin hyperpigmentation despite the lack of sunburn.
  12. Diarrhea, which may be greasy.
  13. Nausea
  14. Urine changes color to darker.
  15. Small yellow or white bumps under the skin or around the eyes.
  16. Unexplained weight loss.

Causes and risk factors of primary biliary cirrhosis

The cause of primary biliary cirrhosis

There is no known cause of this disease. Like other autoimmune diseases, the body suddenly starts attacking its cells and making them defective.

It is worth noting that primary cirrhosis is usually associated with other autoimmune diseases, notably:

  1. Thyroid diseases.
  2. Autoimmune hepatitis.
  3. Scleroderma.
  4. Raynaud’s disease.
  5. Sjögren’s syndrome

Factors that increase the risk of developing primary biliary cholangitis

Listed below are five factors that increase the risk of developing primary biliary cholangitis:


Health professionals have found that this disease affects women more than men. 90% of the total infections are women. Medical estimates indicate that women with inflammation of the first bile ducts range from 65 out of 100,000 women, while that of men ranges from 12 out of 100,000.


The picture shows the different stages that
the liver goes through before
reaching cancer stage

The disease is more common in the age group between 35-60 years than in other age groups.


Experts believe that genetics have a role in this disease. So more than one family member can catch the disease.

Ethnic origins

Doctors have found that people of African descent suffer from this disease more than others.

Other factors

These factors are smoking and exposure to chemicals.

Complications of primary cirrhosis

The most common complications of primary biliary cirrhosis are:

Urinary tract infection

People with primary biliary cholangitis frequently get a urinary tract infection. The infection recurs after each treatment.

Sometimes this inflammation extends to the kidneys, causing many serious health problems.


A person with primary biliary cholangitis suffers from osteoporosis. Very often, the softness of the bones leads to frequent fractures.

Portal hypertension

Blood travels from the intestine, spleen, and pancreas to the liver through a large blood vessel called the portal vein. When scar tissue from cirrhosis of the liver prevents normal blood flow through the liver due to primary cholangitis, this increases pressure within the vein, causing many health problems.

Other complications

Other complications of primary biliary cirrhosis are:

  1. Hypothyroidism.
  2. High levels of cholesterol in the blood.
  3. Lack of fat-soluble vitamins.
  4. Liver cancer: it occurs in stages that are too advanced to be treated.
  5. Gallstones.
  6. Cirrhosis of the liver;
  7. Hepatic encephalopathy.
  8. Gastrointestinal disorders.

Diagnosis of primary cholangitis

The methods of diagnosing primary biliary cholangitis are:

Liver in the stage of primary bliary cirrhosis
Cirrhotic liver

Physical examination

The doctor conducts a clinical examination of the patient. He asks him about the symptoms he suffers from, and about his family’s medical history. Then, he recommends a set of tests related to the liver.

General liver examinations

All or some of the following liver tests are performed, if the result of one of the tests is higher than normal. Then, we say that the body suffers from liver disease. The following are the tests that the doctor relies on to determine if a patient has liver disease or not:

  1. Alanine aminotransferase (ALT).
  2. Aspartate transaminase (AST).
  3. Bilirubin;
  4. Gamma-glutamyltransferase (GGT) enzyme.
  5. Lactate dehydrogenase (LD) enzyme.
  6. Prothrombin time (PT).
  7. Alkaline phosphatase (ALP).
  8. Albumin and total protein

A blood test to check for the presence of mitochondrial antibodies (AMAs).

A blood test to check for the presence of mitochondrial antibodies is a test that largely detects the disease. These antibodies to mitochondria are found only when one is infected by this disease.

Nevertheless, in some rare cases,  this test may not show these mitochondrial antibodies. Despite the effectiveness of the test, primary biliary cholangitis may not be detected.

Ultrasound imaging

Ultrasound imaging enables doctors to see if there are any abnormal results in the liver. But the problem with this diagnosis is that it is not specific to primary biliary cholangitis. Rather, it is a general examination of the health of the liver. it can be used from time to time to find out what pathological changes the liver undergoes over time.

Primary biliary cholangitis treatment

There is no known cure for primary biliary cholangitis. The treatment prescribed by the doctor aims at alleviating symptoms and complications and delaying cirrhosis of the liver for as long a time as possible. So in the remaining part of this article, you will explore the different treatments for these symptoms and complications:

Treatment of hepatic jaundice

The doctor prescribes medications that move the hepatic bile laden with toxins out of the liver. One of the most well-known of these drugs is Ursodeoxycholic acid.

Cirrhosis disease

Treatment of severe itching

To treat this symptom, the doctor prescribes each of the following medicines:

  1. Pain killers.
  2. Anti-itch drugs, notably: Hydroxyzine and Cholestyramine.

Osteoporosis treatment

The doctor treats osteoporosis in the following ways:

  1. By recommending regular exercise.
  2. By prescribing some medications that contribute to the treatment of the symptom, including Alendronate and Risedronic acid.

The ultimate treatment

In case the liver has reached an advanced stage of cirrhosis and no medication can alleviate the condition, the proposed medical solution is liver transplantation. In this operation, part of the liver can be taken from a living person, or the entire liver is taken from a deceased person for transplantation.

Prevention of primary biliary cholangitis

Because primary biliary cholangitis is an autoimmune disease of an unknown cause, its prevention is difficult. But following certain healthy patterns can reduce its severity. The most prominent of these patterns are:

  1. Quitting smoking.
  2. Avoid alcohol consumption completely.
  3. Stopping the use of drugs and undergoing intensive drug addiction treatment.
  4. Taking medications exactly as prescribed by the doctor.
  5. Eating healthy foods that are rich in each of the following vitamins and minerals, especially: Vitamin A, Vitamin D, Vitamin K, Vitamin E, Calcium, Potassium, and Sodium.
  6. Staying away from harmful foods, especially processed and fried ones.
  7. Exercising regularly for at least 30 minutes daily.
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