• Drug treatment

For the treatment of primary biliary cirrhosis are used: immunosuppressants, corticosteroid hormones, drugs that enhance the excretion of copper, ursodeoxycholic acid.

• Ursodeoxycholic Acid (UDCA).

Treatment of primary biliary cirrhosis with ursodeoxycholic acid ( Ursofalk , Ursosan ) should be started already at the first stage (stage of inflammation) or, at least, at the second stage (stage of progressive inflammation) of the disease.

UDCA has a positive effect on such prognostic factors of primary biliary cirrhosis as: the level of serum bilirubin, the severity of liver tissue necrosis, the progression of histological changes, ascites and edema, as well as the severity of skin itching and the intensity of general weakness.

Against the background of the use of ursodeoxycholic acid, indications for liver transplantation are less likely to occur, and the life expectancy of patients increases. At stages I and II of primary biliary cirrhosis, the life expectancy of patients is 20–25 years, therefore, the therapeutic effectiveness of UDCA in the late stages is markedly reduced.

With the use of UDCA at a dose of 10-15 mg / kg / day, the level of laboratory parameters characteristic of primary biliary cirrhosis - alkaline phosphatase, gamma-glutamyl transpeptidase, immunoglobulin M and aminotransferases significantly decreases (compared with the initial level).

In approximately 20% of patients, treatment with the drug may be ineffective.

• Immunosuppressants.

Immunosuppressants inhibit immune responses and prevent disease progression.

Methotrexate (Methotrexate-Ebeve) improves the biochemical and histological parameters of the disease. It is administered orally at a dose of 15 mg per week.

Cyclosporin ( Sandimmun-Neoral ) is administered orally at a dose of 3 mg / kg / day (in 2 divided doses).

• Anti-inflammatory therapy.

Nonsteroidal anti-inflammatory drugs and corticosteroid hormones can be used in low doses. Not prescribed during pregnancy and lactation.

Corticosteroid hormones can reduce the severity of clinical manifestations of primary biliary cirrhosis and improve biochemical and histological parameters of the disease. When prescribing, it is necessary to remember the possibility of developing osteoporosis during treatment.

Prednisoloneum ( Prednisoneumum table. ) Is appointed inside at a dose of 30 mg / day. Within 8 weeks, the dose is reduced to a maintenance dose of 10 mg / day.

• Treatment of copper metabolism disorders.

In patients with primary biliary cirrhosis, copper metabolism is impaired. Therefore, it is necessary to use drugs that contribute to its removal from the body.

The drug of choice is D-penicillamine ( Cuprenyl ). It is a copper chelator. It is applied orally, on an empty stomach (one should not eat food 1.5 hours before and after ingestion). Adults are prescribed with a gradual increase in dose from 250 mg / day (up to 2 g per day). The dose is divided into 4 doses. The dose is increased by 250 mg per week.

• Itchy skin treatment.

Itchy skin is often resistant to therapy and significantly impairs the quality of life of patients with primary biliary cirrhosis.

• Colestyramine.

Cholestyramine (Questran) increases the excretion of bile acids, preventing their absorption. It is prescribed in a dose of 4 mg (mixed with water) during or after a meal; the daily dose is 12-16 mg. Colestyramine should be taken 1.5 hours before or after taking other drugs to avoid non-specific binding and reduce their absorption in the intestine. With a decrease in itching, the dose of colestyramine should be reduced to a minimum.

• Colestipol.

The drug forms a soluble complex between low density lipoproteins and bile acids, helping to eliminate the latter. It is prescribed orally: in granules - at a dose of 5-30 mg / day (2-4 r per day), gradually increasing it every 1-2 months by 5 g; in tablets of 2-16 mg / day at the beginning of treatment, increasing the dose by 2 g every 1-2 months.

• Rifampin

Rifampin (Benemycin, Rimactan, Tibitsin) is used orally at a dose of 300 mg / day 2 r / day. The drug induces microsomal liver enzymes, inhibits hepatocyte uptake of bile acids and facilitates the process of their excretion. It is prescribed to patients who have intolerance to colestyramine.

• Antihistamines.

Appointed at an early stage of the disease. Use with caution in the presence of cirrhosis and signs of hepatic encephalopathy (drugs can inhibit brain function).

Hydroxyzine ( Atarax ) is prescribed orally, at a dose of 25-100 mg 2-4 r / day.