Primary biliary cholangitis, formerly called primary biliary cirrhosis, is a rare and chronic disease of the liver that causes inflammation and progressive destruction of the bile ducts, which are small channels that carry bile from the liver to the gallbladder and intestine.
Although its causes are still unclear, it is considered an autoimmune disease and influenced by genetics, which mainly affects women aged 40 to 60 years.
Although there is no cure for biliary cholangitis, if treatment is started early, it is possible to delay the development of the disease, preventing the destruction of the bile ducts from leading to the impounding of bile, the formation of an inoperative scar tissue and the development of cirrhosis. of the liver. Check out how to identify cirrhosis of the liver.
Main symptoms
In most cases, primary biliary cholangitis does not cause any symptoms and, therefore, in many cases, the disease continues to develop until it is discovered in routine exams or even seriously compromises the liver. At this stage, it can cause symptoms such as:
- Excessive tiredness; Itchy skin; Dry eyes and mouth; Muscle and joint pain; Swelling of the feet and ankles; Yellow skin and eyes; Diarrhea with fatty mucus.
It is also common for primary biliary cholangitis to appear in association with other autoimmune diseases, such as dry keratoconjunctivitis, Sjogren's syndrome, rheumatoid arthritis, scleroderma or Hashimoto's thyroiditis, for example.
As the disease is related to genetics, people who have cases of this disease in the family can have tests to identify whether they also have biliary cholangitis, because, although it is not an inherited disease, there are more chances of having several cases within the same family.
How to diagnose
Generally, suspicion of primary biliary cholangitis arises when changes are seen in a routine blood test done to assess liver function, such as increased liver enzymes or bilirubin.
In these cases, in order to identify the disease, the doctor may order other more specific tests such as dosage of anti-mitochondrial antibodies, anti-nuclear antibodies and markers of bile lesions such as alkaline phosphatase or GGT.
Imaging tests, such as ultrasound or cholangiography, may be indicated to assess liver structures. In addition, a liver biopsy may also be necessary if there are doubts about the diagnosis or to assess the evolution of the disease. Find out more about the liver tests.
How the treatment is done
The goal of treating primary biliary cholangitis is to control the symptoms and prevent the disease from getting worse, so the doctor may recommend the use of remedies such as:
- Ursodeoxycholic acid: is the main medicine used in the treatment and helps bile to leave the liver, preventing the accumulation of toxins in the liver; Cholestyramine: it is a powder that must be mixed in food or drink and that helps to relieve the itchiness caused by the disease; Pilocarpine and moisturizing eye drops: helps to hydrate the mucous membranes of the eyes and mouth, preventing dryness.
In addition to these, the doctor may recommend other medications, according to the symptoms of each patient. In addition, in the most severe cases, it may still be necessary to have a liver transplant, especially when the damage is already very advanced.
Obeticolic acid is a new drug that may help in the treatment of these patients, with the function of improving the flow of bile, preventing inflammation and obstruction of the bile ducts, however, so far it is not yet being marketed in Brazil..
