• Surgery

  • Transjugular intrahepatic portosystemic shunting .

This procedure is performed with frequent bleeding from varicose veins of the esophagus. Portosystemic shunting leads to rapid decompression of the portal system, can significantly reduce ascites .

• Liver transplantation .

Factors that indicate the progression of primary biliary cirrhosis are: increased levels of bilirubin and albumin, lengthening of prothrombin time. The decompensation indicators of primary biliary cirrhosis are also: therapy-resistant skin itching and ascites, bleeding from varicose veins that do not stop after endoscopic treatment, hepatic encephalopathy. In these cases, a decision is made on liver transplantation.

One-year survival after liver transplantation is> 90%; 5 year old> 80%; 10 year old - 65%.

AMA can be found in the blood of patients after transplantation. Relapse of primary biliary cirrhosis during the first few years after surgery is observed in 15% of patients; after 10 years, this figure rises to 30%.

Relapse of primary biliary cirrhosis after liver transplantation: periportal fibrosis and damage to the bile ducts are visualized.

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• Treatment of complications of primary biliary cirrhosis

It is important to begin treatment of complications of primary biliary cirrhosis as early as possible (osteoporosis, multivitamin deficiency, hypothyroidism, and esophageal motility disorders).

• Treatment of multivitamin deficiency.

Intramuscular vitamin A ( retinol ) is prescribed for 1 million units and vitamin K ( phytomenadion ) at 10 mg. With a deficiency of vitamin E, patients undergo substitution therapy (100 mg 2 times / day). The appointment of vitamin D ( cholecalciferol ) at 50,000 units 1-2 times a week is recommended .

• Treatment of osteoporosis.

The appointment of vitamin D at 50,000 units 1-2 times a week is recommended. Bisphosphonates ( raloxifene ) are used.

• Treatment of hypothyroidism.

Treatment of hypothyroidism is carried out by thyroxine.

• Treatment of disorders of the motility of the esophagus.

The tactics of treatment depend on the degree of activity of reflux esophagitis .

 

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• Tactics

From the moment of the first detection of antimitochondrial antibodies (manifestation of the disease) until the diagnosis is established, an average of 6-8 years passes.

The time from the moment of diagnosis to the stage is from 4 to 6 years. In the next 4 years, 20% of patients develop decompensated cirrhosis.

The average length of the period from compensated cirrhosis to the development of the terminal stage of the disease is about 4 years. Thus, from the time of diagnosis to death, approximately 12-14 years pass, and from the probable onset of the disease (the moment of detection of antimitochondrial antibodies) to the time of death - 20-22 years.

Treatment of primary biliary cirrhosis must be started already at stage I (stage of inflammation) or, at least, at stage II (stage of progressive inflammation) of the disease.

With the development of liver cirrhosis and portal hypertension , ascites , varicose veins of the esophagus and stomach are treated.

Patients at a late stage of primary biliary cirrhosis receive an ultrasound every 6 months to exclude the occurrence of malignant neoplasms. Hepatocellular carcinoma is found in 6% of patients with primary biliary cirrhosis, more often in men.