Primary biliary cirrhosis

Introduction

Introduction to primary biliary cirrhosis

Primary Biliary Cirrhosis (PBC) is a chronic intrahepatic cholestatic disease. Serum anti-mitochondrial antibody (AMA) is a specific marker for the diagnosis of PBC. Ursodeoxycholic acid (UDCA) is the only randomized A safe and effective drug for the treatment of PBC as demonstrated by clinical trials. Although the pathogenesis of PBC may be related to autoimmunity, the efficacy of immunosuppressive agents has not been confirmed, and drug-related adverse reactions have limited its clinical application. PBC's condition is progressive, eventually leading to cirrhosis and liver failure. Liver transplantation is the only effective treatment for patients with end-stage PBC.

basic knowledge

The proportion of illness: 0.002%

Susceptible people: middle-aged women

Mode of infection: non-infectious

Complications: pruritus Osteoporosis

Pathogen

Cause of primary biliary cirrhosis

The current cause is not clear and may be related to autoimmunity.

Prevention

Primary biliary cirrhosis prevention

Prevention and treatment of complications of refractory cholestasis.

Complication

Primary biliary cirrhosis complications Complications pruritus osteoporosis

Many patients have elevated serum bilirubin with autoimmune disorders. It can also cause itching, osteoporosis, portal hypertension, and thyroid disease.

Symptom

Symptoms of primary biliary cirrhosis Common symptoms Hepatic atrophy Portal venous stasis Hepatic stellate cell hyperplasia Biliary cirrhosis Liver mild steatosis Gallstones Skin sclera Yellow stained Ascites Fat effusion Gallbladder enlargement

History and symptoms

The disease often coexists with other immune diseases such as rheumatoid joints, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis, etc., more common in middle-aged women, insidious onset, after slow, early symptoms are mild, The patient is generally in good condition, and the appetite and body weight are not significantly decreased. About 10% of the patients may have no symptoms. For patients with chronic obstructive jaundice of unknown cause, especially those with steatorrhea, the cause of the onset should be detailed. And the progress of the disease, whether there are other immune diseases, pay attention to the identification of jaundice with secondary biliary cirrhosis and other causes of cirrhosis.

Experience discovery

Skin, sclera yellow staining, visible multiple scratches and desquamation, liver, splenomegaly surface is still smooth, no tenderness.

Auxiliary inspection

Blood lipids, serum bile acid, combined with bilirubin, AKP and GGT and other microbiliary enzymes were significantly elevated, normal or light, mild, moderately elevated, anti-mitochondrial antibodies in the blood, elevated IgM, prolonged prothrombin time, Urinary bilirubin is positive, urinary bile is normal or reduced.

Imaging ultrasound, ERCP, CT, PTC, etc. to understand whether there are intrahepatic and extrahepatic bile duct dilatation and diseases that cause extrahepatic obstructive jaundice.

Examine

Examination of primary biliary cirrhosis

Blood lipids, serum bile acid, combined with bilirubin, AKP and GGT and other microbiliary enzymes were significantly elevated, normal or light, mild, moderately elevated, anti-mitochondrial antibodies in the blood, elevated IgM, prolonged prothrombin time, Urinary bilirubin is positive, urinary bile is normal or reduced.

Imaging ultrasound, ERCP, CT, PTC, etc. to understand whether there are intrahepatic and extrahepatic bile duct dilatation and diseases that cause extrahepatic obstructive jaundice.

Diagnosis

Diagnosis and diagnosis of primary biliary cirrhosis

Diseases to be diagnosed include: extrahepatic biliary obstruction, chronic active hepatitis, primary sclerosing cholangitis, drug-induced cholestasis. Extrahepatic biliary obstruction that may be cured should be excluded as soon as possible. Ultrasound and ERCP in some cases A check is also necessary. A liver biopsy can confirm the diagnosis, but it is often non-specific. In a few cases, a diagnostic laparotomy is required.

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