Secondary biliary cirrhosis of the liver

Secondary biliary cirrhosis of the liver is cirrhosis that develops as a result of prolonged disruption of bile outflow at the level of large intrahepatic bile ducts.

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Causes secondary to biliary cirrhosis.

The main etiological factors of secondary biliary cirrhosis of the liver:

• congenital defects of the extrahepatic bile ducts (atresia, hypoplasia) are the most common cause of secondary biliary cirrhosis in young children;
• cholelithiasis;
• postoperative stenosis of the bile ducts;
• benign tumors;
• Malignant tumors are rarely the cause of secondary biliary cirrhosis of the liver, since death of patients occurs before cirrhosis develops. However, with cancer of the head of the pancreas, large duodenal papilla, bile ducts, the development of secondary biliary cirrhosis is possible due to the lower growth rates of these tumors;
• compression of the bile ducts by enlarged lymph nodes (with lymphogranulomatosis, lymphocytic leukemia, etc.);
• common bile duct cysts;
• ascending purulent cholangitis;
• primary sclerosing cholangitis.

The development of secondary biliary cirrhosis of the liver is caused by partial, often recurring obstruction of bile outflow. Complete obstruction of the bile ducts is either eliminated surgically or (if surgery is not performed or is impossible) leads to death before the development of biliary cirrhosis of the liver.

The main pathogenetic factors of secondary biliary cirrhosis of the liver are:

• initial cholestasis;
• the entry of bile components into the periductal spaces due to biliary hypertension due to mechanical obstruction of the biliary tract;
• perilobular fibrosis.

The complete histological picture of liver cirrhosis does not always develop. Autoimmune mechanisms do not participate in the development of secondary biliary cirrhosis of the liver.

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Symptoms secondary to biliary cirrhosis.

The clinical symptoms of secondary biliary cirrhosis are primarily determined by the primary pathological process that led to the development of biliary cirrhosis. However, there are also general clinical signs.

1. Intense itching of the skin is often the first clinical manifestation.
2. Intense jaundice develops quite early, gradually increasing even more. It is accompanied by the appearance of dark urine (due to bilirubinemia) and discoloration of feces (acholia).
3. In most patients, the appearance of jaundice is preceded by intense pain syndrome (pain in the right hypochondrium is most common in cholelithiasis, malignant tumors, and in the presence of a stone, the pain is paroxysmal, while in the case of a malignant tumor, it is constant).
4. Quite often there is infectious cholangitis, accompanied by an increase in body temperature to 39-40°C, chills and sweating.
5. The liver is enlarged, dense, and significantly painful upon palpation (during exacerbation of cholelithiasis, in the presence of infectious cholangitis).
6. The spleen is enlarged in the early stages of the disease in the presence of infectious cholangitis; in other cases, splenomegaly is observed at the stage of established liver cirrhosis.
7. In the late stages, classic signs of liver cirrhosis are formed - manifestations of hepatocellular insufficiency and portal hypertension. According to A. F. Bluger (1984), secondary biliary cirrhosis of the liver is formed in children with congenital atresia of the biliary tract within 2 to 5 months, in adult patients with malignant obstruction of the biliary tract - 7-9 months (sometimes after 3-4 months), with obstruction by a stone - up to 1-2 years, with strictures of the common bile duct - 4-7 years.

Diagnostics secondary to biliary cirrhosis.

1. Complete blood count: anemia, leukocytosis, left shift in leukocyte count (especially pronounced in infectious cholangitis).
2. General urine analysis: proteinuria, the appearance of bilirubin in the urine, which causes the dark color of the urine.
3. Biochemical blood test: hyperbilirubinemia (due to the conjugated fraction of bilirubin), decreased albumin content, increased -a2- and beta-globulins, less often gamma-globulins, increased thymol and decreased sublimate test.
4. Immunological blood test: no significant changes. Antimitochondrial antibodies (pathognomonic sign of primary biliary cirrhosis of the liver) are absent.
5. Ultrasound of the liver and biliary tract: enlarged liver, presence of an obstruction to the outflow of bile in the large biliary tract (hepatic ducts, common hepatic duct, common bile duct) in the form of a stone, tumor, etc.
6. Retrograde cholangiography: presence of an obstruction to the outflow of bile in the biliary tract.
7. Liver puncture biopsy (performed specifically during laparoscopy): proliferation of cholangioli, inflammatory infiltration of portal tracts. Unlike primary biliary cirrhosis, centrilobular cholestasis is characteristic with accumulation of bile in the cytoplasm of centrilobular hepatocytes and in the lumen of bile capillaries, destruction of interlobular and small bile ducts is absent. Neutrophilic leukocytes predominate in the infiltration of portal tracts. The lobular structure of the liver may remain normal, regeneration is poorly expressed, periductal fibrosis develops.

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