Primary biliary cirrhosis of the liver

This class of human diseases, as autoimmune, is associated with impaired immune system functions and pathological production of autoimmune antibodies that act against healthy body tissues and lead to their inflammatory alteration or destruction. These pathologies can affect various organs and systems, including the liver. Thus, in women, especially at the age of 40-50 years, primary biliary cirrhosis of the liver can develop, and in many cases the family character of the disease is noted (among sisters, mothers and daughters, etc.).

Causes and stages of primary biliary cirrhosis

At the moment, it is not known exactly what is the trigger mechanism for the development of primary biliary cirrhosis, on this issue studies and discussions are under way. Among the assumptions about the causes of pathology are the following:

• viral and bacterial infections (recently theories about the infectious nature of the disease are highly questioned);
• hormonal disorders;
• genetic factors;
• impact on the body of chemicals;
• other autoimmune diseases (rheumatoid arthritis, Raynaud's disease, Sjogren's syndrome, autoimmune thyroiditis, scleroderma, tubular renal acidosis, etc.).

There are four stages in the development of the disease:

1. At the initial stage, as a result of autoimmune reactions, a non-inflammatory inflammatory inflammation of the intrahepatic bile ducts occurs, bile stagnation is observed.
2. Then there is a decrease in the number of bile ducts, blockade of excretion of bile and its entry into the blood.
3. The portal tracts of the liver are replaced with scar tissue, signs of active inflammation and necrotic phenomena in the parenchyma are observed.
4. Stage of small- and coarse-nodular cirrhosis with signs of peripheral and central cholestasis.

Symptoms of primary biliary cirrhosis

The first manifestations of pathology, to which patients most often complain, are:

• severe weakness, fast fatigue, increased drowsiness, significantly limiting activity and impairing ability to work;
• skin itching (in many cases pronounced, worse at night, and after taking a bath, touching woolen products);
• thickening of the skin, strengthening the skin pattern;
• icterus of the skin, mucous membranes, sclera;
• dry eyes ;
• discomfort, tenderness in the right hypochondrium.

Also, patients are often disturbed by a slight increase in body temperature, headache, lack of appetite, weight loss, depressive condition. In some patients, the primary biliary cirrhosis in the initial stage of compensation is virtually asymptomatic.

Then the following symptoms are added to the listed symptoms:

• Vascular sprouts on the skin;
• redness of the face, feet and palms;
• deformation of nails ("watch glass"), the appearance of white transverse bands on them;
• thickening of nail phalanges.

Because of the disruption of the absorption of vitamins and other nutrients, osteoporosis, steatorrhea, hypothyroidism, varicose veins of the hemorrhoid and esophageal veins, ascites, increased bleeding and other complications can also form.

Diagnosis of primary biliary cirrhosis

The delivery of this diagnosis is based on laboratory tests:

• general blood analysis;
• biochemical blood test ;
• coagulogram;
• hepatic assays;
• lipidogram;
• general urine analysis.

Confirm the diagnosis is possible through a liver biopsy, which is carried out under ultrasound control.

Treatment of primary biliary cirrhosis

Specific treatment of the disease does not exist, only methods that reduce the severity of clinical symptoms, stop the progression of cirrhosis, prevent the development of severe complications. Basically, these are medicamentous schemes with the appointment of immunosuppressive drugs, glucocorticosteroids, cholagogues, hepatoprotectors, antihistamines, etc. Physiotherapeutic methods are also used, a special diet is prescribed. In severe cases, surgical interventions are carried out right up to the liver transplantation.