Biliary cirrhosis - A chronic, progressive disease characterized by the gradual replacement of liver tissue by fibrous scar. Pathology long time not seen often diagnosed incidentally during screening examinations. In the later stages characterized by external signs (small signs of liver), portal hypertension, the defeat of other organs and systems, hepatic encephalopathy. Preliminary diagnosis of cirrhosis and its complications is established on the basis of auxiliary examination methods: ultrasound, CT scan of the hepatobiliary system, ERCP, clinical and biochemical analyzes. The definitive diagnosis is made by blood and liver biopsy detection of mitochondrial antibodies. The disease is incurable, in the later stages to save the patient's life can only liver transplantation. Nonsurgical treatment consists of symptomatic therapy, detoxification, vitamin therapy and dieting.
Primary biliary cirrhosis is not fully understood, so unknown, and the exact reasons for its occurrence. More than 95% of cases of the disease in the blood are determined by mitochondrial antibodies. However, scientists still can not figure out why only affects the mitochondria of liver tissue, and other tissues do not. Intrigue of the disease in its early hidden - often biochemical signs of the disease can be detected by chance during examination for other diseases. The disease progresses to advanced stages of liver damage shows its transplant - only in this case it is possible to save the life of a sick person.
gastroenterology associates the development of primary biliary cirrhosis with autoimmune disorders in the body. The disease is a hereditary nature: biliary cirrhosis cases are very frequent in the family circle. On the autoimmune nature of the disease and says its relationship with other diseases in this group: rheumatoid arthritis, renal tubular acidosis, Sjogren's syndrome, autoimmune thyroiditis, and others.
Although the detection of antibodies to mitochondria in virtually all patients with this form of cirrhosis, detect the connection of these antibodies with the destruction of liver cells and failed. It is known that the disease begins with aseptic inflammation of the biliary tract, severe progressive epithelial proliferation and fibrosis in the ducts. Gradually the inflammation subsides, but fibrosis continues to spread to the liver tissue, causing it to irreversible destruction.
Provoke the beginning of the process and other diseases: congenital malformations and acquired deformities of the biliary tract, cholelithiasis, intrahepatic tumor and enlarged lymph nodes in the liver, bile duct cysts, primary sclerosing cholangitis, or purulent. Also provoke the onset of disease infection can Gram - enterobacteria, which are on the surface similar to mitochondrial antigens. The blood in this disease are determined not only antibodies to mitochondria, and smooth muscle cells, but other immune disorders: increased levels of immunoglobulin M, violation of production of B- and T-lymphocytes. All this speaks in favor of an autoimmune pathogenesis of primary biliary cirrhosis.
Secondary biliary cirrhosis develops in the background of diseases, leading to stagnation of bile in the extrahepatic biliary tract. These include atresia, stones and strictures of the bile ducts, extrahepatic biliary tract cancer, choledochal cysts and cystic fibrosis.
ascites, encephalopathy, bilirubin numbers, albumin and prothrombin index (a measure of blood coagulation system). Depending on the amount of points assigned for these indicators distinguished stage cirrhosis compensation. Class A is characterized by a minimum number of points, the life of up to 20 years, survival after abdominal operations, more than 90%. In Class B, life expectancy is less, and mortality after abdominal operations for more than 30%. In the classroom With life expectancy of no more than 3 years, and mortality after abdominal operations is greater than 80%. Assigning patient class B or C, suggesting the need of liver transplantation.
In Russian METAVIR scale widely used in which the degree of fibrosis is assessed based on biopsy data, gradation level - from 0 to 4; 4 level indicates the presence of cirrhosis.
drowsiness, dryness of the eyes. Occasionally the first sign of disease may be heaviness and pain in the right upper quadrant. The examination revealed elevated levels of gamma globulin, a violation of the ratio of AST to ALT, a tendency to hypotension.
As the other signs of disease appear. Outwardly manifested cirrhosis group of small hepatic signs: spider veins on the skin; redness of the face, hands and feet; increasing salivary glands; yellowness of the skin and all mucous membranes; flexion contracture of the hands. Modify and patient nails - are wide and flat (deformation of the nail according to the "hour glass"), they appear whitish transverse bands; nail thickening phalanx ( "drumsticks"). Around the joints and on the face, eyelids are formed whitish nodules - fat.
In addition to the external manifestations, symptoms of portal hypertension appear: by increasing the pressure in the portal vein of the spleen increases in size, in the veins of the abdominal cavity there is stagnation - formed varicose hemorrhoids and esophageal veins in the anterior abdominal wall is formed extended venous network of the type "jellyfish head ". Venous congestion leads to propotevanie fluid in the abdominal cavity with the formation of ascites, in severe cases - peritonitis.
The more pronounced liver damage, the higher the likelihood of cirrhosis complications. Due to malabsorption of vitamin D in about one third of patients have osteoporosis, manifested by pathologic fractures. Malabsorption of fats leads to a deficiency of fat-soluble vitamins (A, K, D, E), which is manifested signs polyhypovitaminosis. Since feces derived excess fat - steatorrhea. In the fifth part of the patients appears accompanying hypothyroidism develop myelopathy and neuropathy. Varicose veins of the esophagus and the rectum can cause massive bleeding to the development of hemorrhagic shock. Liver damage leads to dysfunction of other organs and systems develop hepatorenal, hepato-pulmonary syndrome, gastro and kolopatiya. Due to the fact that the liver does not fulfill the function of purifying the toxins from the body, they are free to circulate in the bloodstream and exert toxic effects on the brain, causing a hepatic encephalopathy. Very often in the final biliary cirrhosis may develop hepatocellular carcinoma (malignant tumor), complete infertility.
biochemical profile. For cirrhosis characterized by increased levels of alkaline phosphatase and r-GTP, offset ALT and AST ratio, the level of bilirubin increased later. We investigate the immune status of the patient: increased levels of immunoglobulin M antibodies to mitochondria, also notes the increasing level of rheumatoid factor, antibodies to smooth muscle fibers, the tissues of the thyroid gland, the nuclei of cells (antinuclear antibodies).
Signs of liver fibrosis can be detected in the liver ultrasound and CT of the hepatobiliary system, but the final diagnosis can be made only after a liver biopsy. Even when biopsy study biliary cirrhosis characteristic changes in the liver can be detected only in the early stages of the disease, then the morphological pattern becomes the same for all types of cirrhosis.
Identification and elimination of diseases, leading to the development of secondary biliary cirrhosis, possibly through additional research: ultrasound of the abdomen, MR cholangiopancreatography, ERCP.
gastroenterologist, hepatologist and surgeon. The objectives of treatment for cirrhosis is to stop the progression of cirrhosis and liver failure elimination phenomena. Slow down the process of fibrosis can use immunosuppressive drugs (inhibit the autoimmune process), cholagogue (eliminate cholestasis). Symptomatic therapy is the prevention and treatment of complications of cirrhosis: hepatic encephalopathy eliminated by dietary therapy (decrease in the proportion of protein, increase vegetable food ingredient), detoxification therapy of portal hypertension reduction achieved using diuretics. Required appointed multivitamin preparations, enzymes are appointed to improve digestion. In cirrhosis of class A and B is recommended to limit the physical and emotional stress, and in class C - complete bed rest.
The most effective method of treatment of biliary cirrhosis is liver transplantation. After transplantation of more than 80% of patients survive five years. Recurrence of primary biliary cirrhosis occurs in 15% of patients, but in most patients the surgical treatment results are excellent.
Prediction and prevention of biliary cirrhosis of the liver
Prevent the appearance of the disease is almost impossible, but significantly reduce its probability can avoiding alcohol and smoking, regular examination by a gastroenterologist in the cases of biliary cirrhosis in the family, good nutrition and a healthy lifestyle.
Prognosis is poor condition. The appearance of the first symptoms of the disease can only be ten years from the onset of the disease, but life expectancy at cirrhosis which began less than 20 years. Prognostically unfavorable is the rapid progression of symptoms and morphological picture, old age associated with cirrhosis of autoimmune diseases. The most unfavorable in terms of the forecast is the disappearance of itching, reduction ksantomatoznyh plaque, lowering cholesterol levels in the blood.