Primary biliary cirrhosis

    The etiology of primary biliary cirrhosis is not yet clear and may be related to autoimmune diseases. Primary biliary cirrhosis often coexists with other immune diseases such as rheumatoid arthritis, Sjogren's syndrome, systemic sclerosis, chronic lymphocytic thyroiditis, etc., and is more common in middle-aged women.

　　Since primary biliary cirrhosis (PBC) may be related to autoimmune diseases, the complications of this disease include the following types:

　　1. Skin itching

　　There is currently no classic and effective treatment for itching of the skin. Oral anion exchange resin cholestyramine is a first-line drug for treating skin itching. If the patient cannot tolerate the side effects of cholestyramine, rifampicin can be used as a second-line medication. Rifampicin can effectively control the itching symptoms of PBC, but it is not effective for all patients. Its effect is often significant after one month of medication. Rifampicin may achieve its antipruritic effect by changing the internal environment of bile acids in liver cells and improving the biochemical indicators of PBC patients. Narcotic drugs such as Nalmephene and Naltrexone can be used for patients who are ineffective to cholestyramine and rifampicin. There are many other methods (such as ultraviolet light, phototherapy, and plasma pheresis therapy, etc.) used for the control of itching symptoms in PBC, but none have been confirmed by regular clinical trials. For intractable itching that cannot be controlled, liver transplantation surgery can be performed.

　　2. Osteoporosis

　　After a clear diagnosis of PBC, regular bone density tests should be conducted, with follow-up every two years thereafter. Patients should be educated to develop good living habits (such as regular sleep and wake times, smoking cessation), and vitamin D and calcium can be supplemented. Post-menopausal female patients are recommended to use hormone replacement therapy, and it is best administered through the skin. If osteoporosis is明显, biophosphonate treatment can be used.

　　About 11% of PBC patients have osteoporosis. Supplementing calcium and vitamin D can prevent demineralization, with recommended doses of 1000 to 1200 mg/d and 25,000 to 50,000 IU, taken twice or three times a week. Recent research has found that raloxifene can prevent bone loss and reduce serum cholesterol. A dose of 60 mg/d, taken continuously for one year, can increase lumbar spine bone mineral density (LS2BMD) without liver toxicity. Additionally, alendronate can effectively increase bone mass.

　　3. SICCA syndrome

　　All patients with PBC should be asked about symptoms such as dry eyes, dry mouth, and difficulty swallowing, and female patients should also be asked about sexual difficulties. If these symptoms are present, appropriate treatment measures should be taken.

　　4. Raynaud's syndrome

　　For patients in cold regions, the management of Raynaud's syndrome is a difficult issue. Patients should avoid exposing their hands and feet to cold environments, and smokers should quit smoking. Calcium channel blockers may be used if necessary, but they may worsen the dysfunction of the lower esophageal sphincter.

　　5. Portal hypertension

　　Patients with PBC can develop pre-sinusoidal portal hypertension before liver cirrhosis, and the treatment of portal hypertension in patients with liver cirrhosis is the same as that for other types of liver cirrhosis. However, the efficacy of beta-blockers for non-cirrhotic pre-sinusoidal portal hypertension needs to be confirmed, and shunt surgery may be considered if necessary. It is recommended that when PBC is diagnosed for the first time, it should be checked for the presence of esophageal varices, and a review should be conducted once every two years thereafter. If varices are found, measures should be taken to prevent bleeding.

　　6. Deficiency of fat-soluble vitamins

　　Hyperbilirubinemia can be associated with deficiencies in fat-soluble vitamins and poor absorption of calcium. In patients without jaundice, little is known about the levels of fat-soluble vitamins and the value of oral supplementation. Fat-soluble vitamin supplementation is best given in water-soluble form. Monthly subcutaneous injection of vitamin K can correct coagulopathy secondary to vitamin K deficiency.

　　7. Thyroid diseases

　　Thyroid diseases can affect about 15% to 25% of patients with Primary Biliary Cholangitis (PBC), and they usually exist before the onset of PBC. It is recommended that when patients are diagnosed with PBC, the levels of serum thyroid hormones should be measured, and regular checks should be conducted.

　　Primary biliary cirrhosis often coexists with other immune diseases such as rheumatoid arthritis, Sjögren's syndrome, systemic sclerosis, chronic lymphocytic thyroiditis, etc., and is more common in middle-aged women. The onset is insidious, the course is slow, the early symptoms are mild, the general condition of the patients is generally good, appetite and weight do not decrease significantly, about 10% of patients may have no symptoms at all. For patients with chronic progressive obstructive jaundice of unknown etiology, especially those with steatorrhea, it is necessary to understand the triggering factors and the course of the disease, whether there are other immune diseases, and pay attention to distinguishing from jaundice due to secondary biliary cirrhosis and liver cirrhosis caused by other reasons.

　　Since the etiology of primary biliary cirrhosis is unknown, there is no effective preventive method for the disease. Try not to eat foods that harm the liver. Maintain the habit of exercise and a positive attitude.

　　Primary biliary cirrhosis is mainly diagnosed clinically based on the following criteria: significantly elevated microbile duct enzymes such as blood lipids, serum bile acids, conjugated bilirubin, alkaline phosphatase, and glutamyl transpeptidase, normal or slightly to moderately elevated transaminases, positive anti-mitochondrial antibodies in the blood, elevated IgM, prolonged prothrombin time, positive bilirubin in urine, and normal or decreased bilirubinogen in urine.

　　Imaging: Ultrasound, endoscopic retrograde cholangiopancreatography, CT, percutaneous liver biopsy cholangiography, etc., to understand whether there is expansion of extrahepatic bile ducts and diseases causing obstructive jaundice outside the liver.

　　The treatment of primary biliary cirrhosis mainly includes adequate rest, high-protein, high-carbohydrate, high-vitamin, low-fat diet, and daily fat intake.