Biliary cirrhosis

Introduction Biliary cirrhosis is cirrhosis caused by biliary obstruction and cholestasis, divided into primary biliary cirrhosis (PBC) and secondary biliary cirrhosis. The latter is caused by long-term obstruction of the extrahepatic bile duct. Primary biliary cirrhosis is generally considered to be an autoimmune disease. Clinically, disease remission and deterioration occur alternately, often accompanied by other autoimmune diseases such as Sjogren's syndrome, systemic lupus erythematosus, rheumatoid joints. Inflammation and chronic lymphocytic thyroiditis. Humoral immunity was significantly abnormal, the anti-mitochondrial antibody positive rate was 90% to 100%, and the titer of 80% of patients was greater than 1:80.

Cause

Secondary biliary cirrhosis

Etiology: Common causes are obstruction of the bile duct system, such as gallstones, tumors (pancreatic head cancer, Vater ampullary carcinoma) and other compression of the extrahepatic biliary tract, causing stenosis and atresia. In children, due to congenital atresia of the extrahepatic biliary tract, followed by cysts of the common bile duct, biliary cirrhosis and so on. This type of cirrhosis can be caused by complete occlusion of the biliary system for more than 6 months.

2. Primary biliary cirrhosis

This disease, also known as chronic non-suppurative destructive cholangitis, is rare in China, the cause is unknown, may be related to autoimmune response. Most occur in middle-aged women. Clinical manifestations include long-term obstructive jaundice, hepatomegaly, and itchy skin caused by bile stimulation. Also often associated with hyperlipidemia and skin xanthomas. There were no obvious lesions in the bile ducts inside and outside the liver. Cavity degeneration and necrosis and lymphocytic infiltration occurred in the interlobular bile duct epithelium in the portal area. Then the bile duct was destroyed and cholestatic occurred. The fibrous tissue proliferated and invaded and separated the hepatic lobules. Developed into cirrhosis.

an examination

Laboratory examination

(1) Increased blood bilirubin: mainly direct bilirubin, urinary bilirubin positive, due to reduced bilirubin excretion from bile, fecal biliary and urinary biliary.

(2) Increased serum alkaline phosphatase: The source of alkaline phosphatase is the bile duct epithelium. Primary biliary cirrhosis can increase the alkaline phosphatase before the clinical symptoms appear.

(3) Increased blood lipids: In particular, the increase in phospholipids and cholesterol is most pronounced, and triglycerides can be normal or moderately elevated.

(4) Liver function test: Increased serum bile acid concentration, prolonged prothrombin time, can return to normal after early injection of vitamin K, and can not be corrected in advanced liver failure. Serum albumin is normal in the early and middle stages of the disease, and decreases in the late stage, and globulin increases.

(5) Immunological examination: The positive rate of anti-mitochondrial antibodies can reach 90% to 100%, which can be used as an important reference for diagnosis. The positive rate of this antibody in chronic active hepatitis is 10% to 25%, and a small number of occult cirrhosis, systemic lupus erythematosus and rheumatoid arthritis can also be positive. One-third of patients had anti-cholangiocyte antibodies, a few had anti-smooth muscle antibodies and anti-nuclear antibodies, and 1/2 patients had rheumatoid factor-positive. Serum IgM can be increased.

The combination of anti-mitochondrial antibodies, alkaline phosphatase and IgM has a diagnostic significance for primary biliary cirrhosis.

2. Cholangiography

Exclusion of extrahepatic biliary obstruction can be performed by intravenous, percutaneous transhepatic cholangiography or endoscopic retrograde cholangiography.

3. Liver biopsy

Live pathological examination, there is a diagnostic value, but if the cholestasis is serious, or bleeding tendency should be carefully considered, if necessary, laparotomy and biopsy.

Differential diagnosis

The differential diagnosis of biliary cirrhosis includes obstructive jaundice, such as common bile duct stones, common bile duct tumor, pancreatic head and bile duct stricture. The main method of identification is cholangiography. Should be identified with the following diseases:

Chronic active hepatitis

Where anti-mitochondrial antibodies are positive, accompanied by biliary stagnation and histological bile duct abnormalities, chronic active hepatitis should be excluded first. Chronic active hepatitis in China is more common than primary biliary cirrhosis, short-term corticosteroid therapy The observation of the effect helps to identify.

2. Sclerosing cholangitis

This disease is rare, mainly involving the bile duct, the above immunological markers are negative, and there are many bacterial infectious fever, biliary tract angiography can help identify.

3. Drug-induced jaundice

Such as chlorpromazine, methyl testosterone, sulfonamide, arsenic preparations. Mostly caused by individual allergies, there is a history of medication, within a few weeks after taking the drug, jaundice can last for several years, often accompanied by elevated blood eosinophils, liver biopsy without typical histological manifestations of primary biliary cirrhosis.

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