Generality
Primary biliary cirrhosis is a chronic autoimmune disease that affects the liver and, more specifically, the bile ducts within it.
In this particular liver disease, the immune system attacks the cells that make up the bile ducts, causing inflammation, scarring and closure, resulting in the onset of intrahepatic cholestasis.
Therefore, this pathology evolves into cirrhosis only in its most advanced stages.
Furthermore, in some cases, primary biliary cirrhosis is associated with other types of autoimmune diseases, such as Sjögren's syndrome, rheumatoid arthritis and autoimmune thyroiditis.
Epidemiology
Primary biliary cirrhosis can affect patients of both sexes, in a rather wide age range. However, it is estimated that more than 90% of cases occur in women aged between 35 and 60 years.
Causes
As mentioned, primary biliary cirrhosis is an autoimmune disorder. More in detail, T lymphocytes attack the cells of the bile ducts, causing chronic inflammation and consequent scarring.
The reason why T lymphocytes carry out this attack, unfortunately, is still unknown. Some argue that this mechanism can be triggered by infectious agents or toxic agents and that there is also the involvement of a genetic component.
Diagnosis
The diagnosis of primary biliary cirrhosis can occur through the execution of different types of examinations, such as:
- Blood tests to determine liver function, in which blood levels of transaminases, gamma glutamyl transferase (or gamma-GT are desired) and alkaline phosphatase are evaluated;
- Blood tests performed to search for specific antibodies, such as anti-mitochondrial antibodies and some anti-nuclear antibodies;
- Abdominal ultrasound;
- MRI scan;
- Abdominal CT scan;
- Liver biopsy.
Symptoms and Complications
In most patients, primary biliary cirrhosis presents no symptoms of any kind during the early stages of development. However, some patients may experience symptoms such as:
- Fatigue;
- Dry mouth;
- Itching (caused by the deposition of bile salts in the skin);
- Conjunctival dryness.
Later, with the continuation of the inflammation, therefore with the progression of the pathology, the following symptoms may also occur:
- Pain in the right upper abdominal quadrant;
- Increased liver volume;
- Jaundice;
- splenomegaly;
- Impairment of fat absorption (due to the low production of bile), with consequent emission of oily stools;
- Increased blood cholesterol levels;
- Deposition of lipids in the skin, resulting from their reduced excretion through bile;
- Lipid malabsorption and malnutrition;
- Liver cirrhosis.
One of the most common complications in women is osteoporosis. In fact, due to cholestasis and liver damage, in the presence of primary biliary cirrhosis we see an altered absorption of exogenous vitamin D and a reduced activation of endogenous vitamin D; both these factors can favor the onset of osteoporosis.
Furthermore, patients with primary biliary cirrhosis are at greater risk of developing liver tumors.
Therapy
The pharmacological therapy of primary biliary cirrhosis is based essentially on the administration of ursodeoxycholic acid (Deursil®, Ursobil®). This active ingredient, in fact, is a bile acid able to regulate cholesterol absorption, therefore, it is able to perform those activities that bile is no longer able to exercise due to the pathology.
Ursodeoxycholic acid is administered orally at the usual dose of 300-600 mg of active ingredient per day.
In addition to therapy with ursodeoxycholic acid, the doctor may decide to prescribe the administration of vitamin supplements, in particular, based on vitamin D.
Furthermore, if deemed necessary, the doctor may also decide to intervene with a pharmacological therapy aimed at treating the symptoms of primary biliary cirrhosis. For example, it is possible to proceed with the treatment of the itching caused by the deposition of bile acids in the skin, through the administration of colestyramine (Questran®).
Finally, in severe cases, it may be necessary to perform a liver transplant .
Being an autoimmune disease, research in the field of primary biliary cirrhosis is being directed towards the use of immunosuppressive drugs for its treatment. However, the results obtained so far have not been the expected ones.
Precautions
Unfortunately, in patients with primary biliary cirrhosis it is almost impossible to completely restore liver function. However, these same patients can take small steps and make some changes in their lifestyle, so as to preserve, as far as possible, any liver function that the disease has not yet compromised.
In this regard, patients suffering from primary biliary cirrhosis should avoid consuming alcoholic beverages, adopt a diet low in sodium and limit, as far as possible, the intake of hepatotoxic drugs.
