What is it about ?
The old name for this disease is ‘primary biliary cirrhosis’.
In primary biliary cholangitis (PBC), the small bile ducts of the liver are chronically inflamed. This inflammation causes destruction of the bile ducts. The bile can then no longer flow out of the liver, it stagnates in the liver, this is called cholestasis. Liver cells are irreversibly destroyed and are replaced by scar tissue. This leads to the liver cirrhosis.
The precise cause of this disease is not known. It is believed to be an autoimmune disease, where a person’s immune system produces substances that destroy cells in their own liver. Often there are no symptoms at the onset of the disease. Once symptoms appear, the life expectancy without a liver transplant is 5 to 10 years.
In whom and how often does it occur?
It is found in 10 to 50 people out of 100,000, and 9 times out of 10, it is women who are affected. The patient begins most often between 40 and 60 years.
How to recognize it?
Most often, the disease develops for many years without causing symptoms or with only a few symptoms, and is discovered by chance. The cirrhosis advanced is accompanied by generalized itching, jaundice and an enlarged liver.
The itching occur all over the body and, at first, only in the evening. This can also happen with an increase in the concentration of female hormones (estrogen) in the blood, for example during hormonal treatment or during pregnancy, or with certain medicines which are broken down by the liver.
Jaundice can be recognized by the yellowing of the whites of the eyes and, at an advanced stage, of the skin as well. The urine is dark.
The enlargement of the liver can cause pain and discomfort in the upper right abdomen. Sometimes the palms of the hands turn red, and small, star-shaped blood vessels appear on the skin. In humans, cirrhosis may be accompanied by breast development (gynecomastia).
Non-specific symptoms are also possible such as tired, joint pain, dry mouth or dry eyes.
In the final stage, the liver will be damaged to the point of no longer functioning. Blood pressure rises in the portal vein, a major blood vessel supplying the liver, leading to serious complications, such as varicose veins in the esophagus which can bleed and fluid build-up in the belly (ascites) . THE’osteoporosis is then also more frequent.
How is the diagnosis made?
On clinical examination, the doctor will notice jaundice and an increase in the size of the liver or spleen. Sometimes there is a buildup of fat in the form of yellowish swellings around the eyelids.
Often the diagnosis is made by chance. An abnormality in the blood may appear before symptoms appear. Depending on the disease activity (active or stable), some blood results are abnormal or bordering on normal. This disease is also characterized by a too high cholesterol.
If the liver test results are abnormal, the doctor will order additional blood tests and at least one ultrasound from the top of the belly. If necessary, he performs a liver biopsy to identify possible cirrhosis and determine its magnitude.
The doctor will also look for a osteoporosis, because it is frequently associated with primary biliary cholangitis.
What can you do ?
There is usually nothing you can do to prevent the disease on your own.
If you have this disease, get enough calcium and vitamin D to prevent bone damage. Avoid consuming alcohol so as not to burden your liver, and also avoid drugs that are broken down by the liver.
What can the doctor do?
To date, no drug has slowed the progression of the disease significantly.
Existing treatments based on ursodeoxycholic acid do not have a marked effect. At the initial stage, they may delay the need for a liver transplant. They have not been proven to be effective in the long term. If necessary, these drugs can be combined with cortisone.
The itching can be treated with anti-itch medicines (antihistamines).
THE’osteoporosis can be treated with bisphosphonates or estrogen-based patches to apply to the skin if you are postmenopausal.
If the liver is so damaged that it cannot function, a liver transplant may be necessary. This is most often carried out between the ages of 60 and 65. This disease is monitored by a liver specialist (hepatologist) using regular blood tests and ultrasounds liver.
Want to know more?
- Preparing for a liver biopsy – CHU Montreal
- Ultrasound here Where to find on this page of Cliniques St Luc UCL
- Ursodeoxycholic acid – CBIP – Belgian Center for Pharmacotherapeutic Information
- Antihistamines – CBIP – Belgian Center for Pharmacotherapeutic Information
- Bisphophonates – CBIP – Belgian Center for Pharmacotherapeutic Information
- Resuming my activities after a transplant – CHU Montreal
- Alcohol, the point on your consumption – Stop or else?
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