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.
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.
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 .
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.
In cases where patients do not have manifestations of the disease, antimitochondrial antibodies are detected in the blood serum , but biochemical parameters of liver function remain within normal values, this indicates the slowly progressive nature of the course of primary biliary cirrhosis. Survival parameters in such patients are close to those in the general population.
In this category of patients, as a rule, 15-20 years elapse from the moment of manifestation of primary biliary cirrhosis (detection of antimitochondrial antibodies in the blood serum) to the terminal stage.
The average life expectancy of patients with asymptomatic primary biliary cirrhosis is 16 years from the moment of diagnosis, and in patients with advanced clinical symptoms this indicator is 7.5 years.
Factors of rapid progression of primary biliary cirrhosis (predictors of rapid disease progression) are:
Marked pathological changes in liver biopsy samples - small focal (step) necrosis.
The prevalence of fibrosis.
The severity of cirrhosis.
The presence of granulomas.
Signs of cholestasis.
The elderly age of patients.
The presence of edema and ascites.
Hepatomegaly.
Hepatic encephalopathy.
Bleeding from varicose veins.
The presence of concomitant autoimmune diseases.
Change in laboratory parameters: bilirubin, albumin, alkaline phosphatase, gamma-glutamyltranspeptidase, immunoglobulin M.
In stage I of primary biliary cirrhosis, clinically severe cirrhosis develops within 5 years in 4% of patients, and in stage III in 59% of patients.
The average duration of transition to stage IV with primary biliary cirrhosis is 25 years for patients with stage I, 20 years for patients with stage II, and only 4 years for patients with stage III.
The most important prognostic factors for primary biliary cirrhosis are: serum bilirubin content and Mayo indicators:
In patients with a serum bilirubin content of 2-6 mg / dl (34.2-102.6 μmol / L), the average life expectancy is 4.1 years.
With a serum bilirubin content of 6–10 mg / dl (102.6–170.1 μmol / L), the average life expectancy is 2.1 years.
In patients with serum bilirubin greater than 10 mg / dL (more than 170.1 μmol / L), the average life expectancy is 1.4 years.
The Mayo Risk Scale is a reliable method for assessing the course of primary biliary cirrhosis, both in vivo and during treatment. Mayo's risk scale provides an opportunity to assess the prognosis of the disease and monitor the effectiveness of treatment. In addition, the Mayo risk scale (Mayo model) allows you to calculate the most optimal time for liver transplantation based on an assessment of 5 indicators (without resorting to an analysis of the histological picture):
The age of the patient.
Serum bilirubin content .
Serum albumin content .
Prothrombin time.
The presence of edema or ascites.
Serum bilirubin content (> 17 μmol / L), serum albumin content (<38 g / L) and the severity of step necrosis are independent prognostic factors of primary biliary cirrhosis.
Preventive measures in patients with primary biliary cirrhosis are reduced to the prevention of disease progression.
For this purpose, treatment of the disease must begin at stage I (stage of inflammation) or, in extreme cases, stage II (stage of progressive inflammation).
In the late stages of the disease, when liver cirrhosis and portal hypertension have developed , preventive measures should be aimed at preventing bleeding from varicose veins. For this purpose, all patients with cirrhosis of the liver undergo esophagogastroduodenoscopy.
If the risk of bleeding is high, then the patient should be prescribed beta-blocker therapy. With intolerance to these drugs, sclerotherapy or ligation of varicose veins is carried out. Subsequently, repeated examinations are carried out every 6 months.
If the sizes of varicose veins are small, then a re-examination is carried out after 2-3 years to assess the dynamics of the progression of the pathological process.
If the patient has no varicose veins, then re-examination is carried out after 3-5 years.