Secondary biliary cirrhosis

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

[1], [2], [3], [4], [5]

Causes of the secondary biliary cirrhosis

The main etiological factors of secondary biliary cirrhosis:

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

To the development of secondary biliary cirrhosis lead to partial, often recurring embarrassment of bile outflow. Complete obturation of the bile ducts is either eliminated by the operative route, or (if the operation is not performed or impossible) leads to death before the development of biliary cirrhosis.

The main pathogenetic factors of secondary biliary cirrhosis are:

• initial cholestasis;
• the entry of bile components into the periductal spaces in connection with bile hypertension due to mechanical obstruction of the bile ducts;
• perilobular fibrosis.

A complete histological picture of liver cirrhosis does not always develop. Autoimmune mechanisms of participation in the development of secondary biliary cirrhosis are not accepted.

[6], [7], [8], [9]

Symptoms of the secondary biliary cirrhosis

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 common clinical signs.

1. An intense skin itch is often the first clinical manifestation.
2. An intensive jaundice develops early enough, gradually even more increasing. It is accompanied by the manifestation of dark urine (due to bilirubinemia) and discoloration of feces (achiolia).
3. In most patients, the appearance of jaundice is preceded by an intense pain syndrome (pains in the right hypochondrium region - the most regular in cholelithiasis, malignant tumors, with the presence of a pain stone paroxysmal, with a malignant tumor - a permanent character).
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, considerably painful on palpation (with exacerbation of cholelithiasis, presence of infectious cholangitis).
6. The spleen is enlarged in the early period of the disease in the presence of infectious cholangitis, in other cases splenomegaly is noted at the stage of the formed cirrhosis of the liver.
7. In the late stages of the formation of classical signs of cirrhosis - the manifestations of hepatic-cell insufficiency and portal hypertension. According to AF Bluger (1984), secondary biliary cirrhosis of the liver is formed in children with congenital atresia of the bile ducts in terms of 2 to 5 months, in adults with malignant obturation of the bile ducts - 7-9 months. (sometimes in 3-4 months), with stone obstruction - up to 1-2 years, with strictures of the common bile duct - 4-7 years.

Diagnostics of the secondary biliary cirrhosis

1. The general analysis of the blood: anemia, leukocytosis, shift of the leukocyte formula to the left (especially expressed in case of infectious cholangitis).
2. The general analysis of urine: proteinuria, the appearance of bilirubin in the urine, which causes a dark color of urine.
3. Biochemical blood test: hyperbilirubinemia (due to the conjugated fraction of bilirubin), decrease in albumin content, increase in α2 and β-globulins, less often in γ-globulins, increase in thymol and decrease in the sulemic test.
4. Immunological analysis of blood: without significant changes. Antimitochondrial antibodies (pathognomonic sign of primary biliary cirrhosis) are absent.
5. Ultrasound of the liver and bile ducts: enlargement of the liver, the presence of an obstruction for the outflow of bile in the major biliary tract (hepatic ducts, common hepatic duct, holedoch) in the form of stone, tumor, etc.
6. Retrograde cholangiography: the presence of an obstruction for the outflow of bile in the biliary tract.
7. Puncture liver biopsy (performed aimingly during laparoscopy): proliferation of cholangiol, inflammatory infiltration of portal tracts. Unlike primary biliary cirrhosis, the liver is characterized by centrolobular cholestasis with accumulation of bile in the cytoplasm of centrolobular hepatocytes and in the lumen of the bile capillaries, the destruction of interlobular and small bile ducts is absent. In the infiltration of portal tracts, neutrophilic leukocytes predominate. The lobular structure of the liver can remain normal, regeneration is poor, and periductal fibrosis develops.

[10], [11], [12]