Primary biliary cirrhosis is not a very common pathological condition, presumably of an autoimmune nature. This disease is characterized by the gradual destruction of the intrahepatic bile ducts, due to which bile and various toxic metabolites are retained in the liver. Gradually, this condition leads to inhibition of the functional activity of this organ, the appearance of fibrous, and then cirrhotic changes. The prognosis for such a pathology directly depends on whether it is accompanied by any clinical manifestations. In the most unfavorable scenario, a sick person over time faces liver failure, which threatens his life.
As we have already said, the overall prevalence of this pathological process is low. Among all forms of liver cirrhosis, the primary biliary form accounts for no more than two percent. According to statistics, the overall incidence of this pathology is about one hundred and thirty cases per million of the population. It is interesting that female representatives are much more likely to encounter such a disease. About eighty percent of all patients are women. Most often, this disease is diagnosed in people between the ages of forty and sixty years. No cases were reported among children and adolescents.
A distinctive feature of primary biliary cirrhosis of the liver is its extremely long course. We have already mentioned that the prognosis directly depends on the presence of concomitant clinical manifestations. As an example, we can say that with an asymptomatic course, the average life expectancy is from fifteen to twenty or more years. In the event that any complaints are still present, liver failure occurs after about seven or eight years.
The question of why primary biliary cirrhosis develops is currently still open. Most scientists are inclined to believe that autoimmune disorders are the basis of its occurrence. In addition, with this pathological process, a clearly expressed hereditary predisposition can be traced. Familial cases of this disease are very often identified. It is also noticed that this pathology often proceeds in conjunction with other autoimmune disorders, for example, with rheumatoid arthritis, dermatomyositis, and so on.
Infectious agents, hormonal disorders, toxic substances entering the body, and much more were considered as provoking factors for the formation of such a disease.
Primary biliary cirrhosis of the liver develops with a sequential change of several stages. The first stage is called ductal . It is characterized by an increase in inflammatory and destructive processes in the area of the bile ducts. However, the tissues surrounding the portal tracts remain intact. The second stage is ductullary . Lymphoplasmacytic infiltration extends to the surrounding parenchyma. In this case, the intrahepatic bile ducts are increasingly destroyed. The third stage is accompanied by the appearance of fibrotic changes in the liver stroma. Liver cells become necrotic , signs of bile stagnation intensify. The fourth stage is spoken of if all morphological markers of cirrhosis are present.
Symptoms of primary biliary cirrhosis
Earlier, we have already said that often such a disease is asymptomatic. About fifty percent of sick people do not even know about the problem they have. In this case, the pathological process can be identified only with the help of laboratory tests. One of the earliest symptoms, indicated by more than seventy percent of patients, is increased weakness.
Another specific complaint is that of itchy skin. A sick person often turns to dermatologists for help, who also cannot always establish its cause. On examination, numerous scratches can be found on the surface of the patient’s body. Over time, usually after several months or even years, symptoms such as jaundice, soreness in the right hypochondrium and enlargement of the liver in size join . Xanthomas and spider veins often appear on the skin of a sick person . In some cases, muscle and joint pain, sensory disturbances from the extremities and much more are added.
At the stage when changes in the liver are pronounced, symptoms such as low-grade fever, even greater weakness and increased jaundice, and stool disorder in the form of diarrhea occur. Signs indicating the development of liver failure are gradually added.
The principles of diagnosis and treatment of the disease
Diagnosis of this disease begins with a biochemical blood test. This analysis reveals an increase in a number of indicators that indicate liver malfunction. An immunological study is mandatory, during which an increase in the titer of antibodies to mitochondria is detected . Ultrasound diagnostics and magnetic resonance imaging are not always informative. Biopsy with subsequent histology of the material obtained is of decisive importance in making a diagnosis.
Treatment for primary biliary cirrhosis consists of the appointment of drugs that have an immunosuppressive and anti-inflammatory effect. An example is glucocorticosteroids and cytostatics . The use of ursodeoxycholic acid is also shown . The rest of the therapy is symptomatic.
Prevention of primary biliary cirrhosis
It is not possible to talk about the prevention of the development of this pathological process due to its unexplained cause. The only recommendation is to see a doctor as early as possible if any concerns arise.