Primary biliary cirrhosis
Introduction
Introduction to primary biliary cirrhosis Primary Biliary Cirrhosis (PBC) is a chronic intrahepatic cholestasis disease. Serum anti mitochondrial antibody (AMA) is a specific indicator for the diagnosis of PBC. Ursodeoxycholic acid (UDCA) is the only safe and effective drug for PBC confirmed by randomized controlled clinical trials. Although the pathogenesis of PBC may be related to autoimmunity, the efficacy of immunosuppressants has not yet been confirmed, and its clinical application is limited due to drug-related adverse reactions. The condition of PBC is progressive, which eventually leads to liver failure due to cirrhosis. Liver transplantation is the only effective treatment for end-stage PBC patients. Basic knowledge Prevalence rate: 0.002% Susceptible group: women above middle age Mode of infection: non infectious Complications: skin pruritus, osteoporosis
Cause
Etiology of primary biliary cirrhosis
At present, the etiology is not clear, which may be related to autoimmunity.
Prevention
Prevention of primary biliary cirrhosis
To prevent and treat the complications of intractable cholestasis.
Complication
Complications of primary biliary cirrhosis complication Skin pruritus Osteoporosis
Many patients have increased serum bilirubin, accompanied by autoimmune disorders. It can also cause skin itching, osteoporosis, portal hypertension, thyroid disease, etc.
Symptom
Symptoms of primary biliary cirrhosis common symptom Lobar atrophy, portal vein blood flow stagnation, hepatic stellate cell hyperplasia, biliary cirrhosis, mild hepatic steatosis Gallstone, skin, sclera, yellow stain, ascites, fat diarrhea, gallbladder enlargement
Medical history and symptoms
This disease often coexists with other immune diseases, such as rheumatoid arthritis, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis, etc. It is mostly seen in middle-aged women. The onset of the disease is insidious. After a slow process, the early symptoms are mild. The patients are generally in good condition, and their appetite and weight have no significant decline. About 10% of the patients may have no symptoms,, For patients with chronic progressive obstructive jaundice of unknown cause, especially those with fatty diarrhea, it is necessary to know the inducement of the onset and the progress of the disease in detail, whether there are other immune diseases, and pay attention to distinguish jaundice from secondary biliary cirrhosis and cirrhosis of other causes.
Experience discovery
The skin and sclera are yellow, and many scratches and desquamation can be seen. The surface of the liver and spleen is still smooth, without tenderness.
Supplementary Examination
Blood lipid, serum bile acid, conjugated bilirubin, AKP, GGT and other micro bile duct enzymes increased significantly, nitrogen transferases were normal or slightly, moderately increased, anti mitochondrial antibody in blood was positive, IgM was increased, prothrombin time was prolonged, urinary bilirubin was positive, and urinary bilirubin was normal or decreased.
Imaging Ultrasound, ERCP, CT, PTC, etc. to understand whether there is expansion of intrahepatic and extrahepatic bile ducts and diseases that cause extrahepatic obstructive jaundice.
Examine
Examination of primary biliary cirrhosis
Blood lipid, serum bile acid, conjugated bilirubin, AKP, GGT and other micro bile duct enzymes increased significantly, nitrogen transferases were normal or slightly, moderately increased, anti mitochondrial antibody in blood was positive, IgM was increased, prothrombin time was prolonged, urinary bilirubin was positive, and urinary bilirubin was normal or decreased.
Imaging Ultrasound, ERCP, CT, PTC, etc. to understand whether there is expansion of intrahepatic and extrahepatic bile ducts and diseases that cause extrahepatic obstructive jaundice.
Diagnosis
Diagnosis and differential diagnosis of primary biliary cirrhosis
The diseases to be differentiated include extrahepatic biliary obstruction, chronic active hepatitis, primary sclerosing cholangitis, and drug-induced cholestasis. Possible extrahepatic biliary obstruction should be excluded as soon as possible. Ultrasound examination and ERCP examination are also necessary in some cases. Liver biopsy can be confirmed, but it is often non-specific. In a few cases, diagnostic laparotomy is required.
