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Abstract: Autoimmune hepatitis is a group based on certain inflammatory autoimmune liver disease, including autoimmune hepatitis (Autoimmune hepatitis, AIH), primary biliary cirrhosis (Primary biliary cirrhosis, PBC), primary sclerosing cholangitis (Primary sclerosing cholangitis, PSC) and other, their common feature is inflammation of the liver pathologic damage in the same time, can be found in serum and liver-related autoantibodies cycle. In recent years, the detection of autoantibodies are more and more the importance of clinical and laboratory, especially for autoantibodies in autoimmune liver disease associated application of new testing programs for the past number of unexplained abnormal liver function or poor treatment Diagnosis and treatment of patients basis. With in-depth understanding of autoimmune liver disease, the emergence of a new diagnosis for the classification of autoimmune liver disease that the overlap syndrome, 17 cases were collected in the clinical diagnosis of AIH / PBC overlap syndrome data analysis is as follows . Number of pages: 2 Page Range :294-295 Key words: autoimmune hepatitis, primary biliary cirrhosis overlap syndrome subject classification: R575.1 [medicine, HEALTH "within SCIENCES" digestive and abdominal dise liver and gallbladder dise Hepatitis] R575.2 [medicine, HEALTH "within SCIENCES" digestive and abdominal dise Liver and gall bladder dis cirrhosis] Related Articles: Topics related references (5) citations (2 )

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wn as primary biliary cirrhosis intrahepatic obstructive biliary cirrhosis. The disease is the major intrahepatic bile duct lesions of non-suppurative inflammation and obstruction. The etiology is still unknown, is generally believed that the disease is an autoimmune disease. To T cell-mediated immune response characterized by bile duct lobular attack. Pathogenic factor on biliary epithelial cell structure of the continued attacks lead to progressive destruction of bile duct disappearance. The continued disappearance of lobular bile duct will cause the signs and symptoms of cholestasis, with the disease progress, the last of cirrhosis and liver failure. The disease often associated with other autoimmune diseases such as rheumatoid arthritis, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis and other co-exist. Second People's Hospital of Jingzhou City Department Gang Zhu Jian liver
Can occur in patients with elevated immunoglobulin (IgM increased especially), and mitochondrial antibody, antinuclear antibody, bile duct cells positive for antibodies and other autoantibodies, have prompted this disease and autoimmune related. People think that the existing basis of this disease is the cause of histocompatibility antigen abnormal immune response.
Hepatomegaly is the main pathology of the disease, the liver weight of up to 1500-2500g, dark green, blunt edges, hardness increases, the surface smooth and slightly uneven. Usually no abnormal gallbladder, bile does not contain moderate intracapsular often colorless clear liquid, intrahepatic and extrahepatic bile ducts were no significant changes in bold tube. The reticuloendothelial cell proliferation, a high degree of swelling of the liver and spleen, and significant fibrosis, splenic sinus splenic intramedullary expansion and proliferation of skin cells. The common bile duct hilar lymph node enlargement can be seen. Kidney swelling, distal convoluted possession of non-iron brown tube. All organs are bile pigment-dyed. Pathological changes in liver tissue can be divided into four:
1 (cholangitis period) mainly lobular septum between the bile duct or bile duct with chronic non-suppurative inflammation, timid tube cavity wall and surrounded by lymphocytes, plasma cells, histiocytes and a few eosinophils invasion, leaving the expanded portal area, about half of the cases are in the lymphoid follicles in portal areas and typical granuloma formation. Liver cells and liver cells in the limiting plate of little damage, structural integrity of hepatic lobule, no bile retention phenomenon. 2 (of small bile duct hyperplasia) is characterized by proliferation of small bile ducts, interlobular bile ducts disappeared, the surrounding inflammatory cells or fibroblasts, so as inflammation around the small bile ducts. Liver cells were normal, but in the inflammatory changes around the portal area of liver cells of cholestatic phenomena. Leaflets around capillary bile duct dilatation, microvilli loss, reduced or edema, mitochondrial swelling. 3 (trace period) to reduce inflammation, left stellate scar and portal area of scar tissue or the portal area to another extension within the hepatic lobule. Cholestasis is more serious. 4 (cirrhosis period) showed focal necrosis of liver cells, portal area expanded and connected to each other fibrous septa separating the lobules and the formation of false lobules and regenerative nodules. When the blood vessel formation pseudolobule distorted.
Clinically, the disease more common in women aged 40-60, male and female incidence ratio of 1:9. Insidious onset, early symptoms are mild, the patient generally in good condition, no significant loss of appetite and weight declined more. About 10% of patients may have no symptoms. Course of the disease was slow, common clinical manifestations are:
Chronic progressive obstructive jaundice: the inflammatory bile duct obstruction can produce incomplete obstructive jaundice. Early stage patients can no jaundice, but because of the blood bile salt concentration, stimulation of peripheral nerves are itchy skin, after a long illness, there rough skin, pigmentation, scaling and thickening, itching appears mostly in the 0.5-2 years after Clinical jaundice occurs only about 25% of patients with jaundice, pruritus simultaneously. Jaundice, dark urine period, fecal lighter color, but gray stool rare.
Fat metabolism disorder: the lack of intestinal bile salts, influence of dietary fat emulsification and absorption, causing steatorrhea. Steatorrhea often affect fat-soluble vitamins A, D, K and calcium absorption, and cause malnutrition. Because the lack of vitamin D and calcium malabsorption, can produce osteomalacia, osteoporosis, and even fractures, patients often feel pain in lower back and ribs, etc. The biliary obstruction, serum total lipids and cholesterol content continued to increase, organizations large amounts of cholesterol and the formation of phagocytic yellow warts. Common warts on the eyelids yellow inner canthal, rare in other parts. When liver failure, cholesterol synthesis disorders, serum cholesterol levels fall, yellow warts can also be reduced or even disappear.
Hepatosplenomegaly: the long-term cholestasis, liver enlargement often significant, largest of the lower edge may be flat umbilical, hard, no tenderness, the surface was smooth or finely granular. Often significantly enlarged spleen.
Mucocutaneous bleeding tendency: possible reasons are: vitamin K and coagulation factor VII deficiency; capillary fragility; hypersplenism induced thrombocytopenia.
Advanced cirrhosis and liver failure in all its manifestations: such as spider angioma, esophageal and gastric varices, ascites and hepatic encephalopathy and so on.
Associated diseases: Common Ownership syndrome, scleroderma, renal tubular acidosis, rheumatoid arthritis, autoimmune thyroiditis and so on.
Laboratory examination revealed that almost all patients with PBC can occur ALP increased, often to alarming levels can be high, and the liver-derived serum 5'-nucleotide enzymes and elevated GGT levels often parallel. Serum transaminase levels can be normal or mildly elevated, but rarely more than 5 times the upper limit of normal over the scope of their volatility is very small, little relationship with the prognosis. The disease is usually early in the PBC, serum bilirubin levels are normal, but with the progress of the disease, 60% of patients will rise, including direct bilirubin and indirect bilirubin can be increased. Bilirubin indicates a poor prognosis.
A recent study suggests, there AMA positive, sooner or later will happen PBC. The study pursued the 29 patients positive for the antibody, but no symptoms of PBC, and normal liver function, underwent liver biopsy suggest, though not diagnostic, but the pathological changes found 24 cases of PBC performance. After 10 years of follow-up, 76% of the patients had clinical manifestations of PBC, 83% had cholestatic laboratory abnormalities. 10 patients with repeated liver biopsy, the results of 2 cases of lesions progress from to , the other 2 cases of lesions progress from to of the remaining 6 cases no pathological changes in progress.
PBC can be significantly increased blood fats, at least 50% of patients, there may be increased blood cholesterol, in a yellow tumor patients, their cholesterol levels up to 26mmol / L. Early in the disease that often appears slightly elevated LDL and VLDL, and HDL increased significantly, which is why although the lipids in patients with PBC was significantly higher, but due to atherosclerosis and the risk of death did not occur according to the increasing of the reason. However, advanced disease, LDL gradually increased, while HDL is reduced. PBC, there is another protective factor is the low hyperlipoproteinemia, elevated lipoprotein is an independent risk factor for coronary artery disease.
Treatment of this disease should include two aspects: Cholestasis caused by chronic symptoms and complications, including itching, control of metabolic bone disease, to correct high cholesterol, vitamin deficiency and anemia, nutritional disorders and to improve the absorption and treatment of thyroid disease and so on. Causative factor for the damage to the lobular bile ducts.the study notes 12:52 15 font: big in the small primary biliary cirrhosis (PBC) is an autoimmune disease occurs in middle-aged women, in most cases when a clear diagnosis no clinical symptoms. Serum AMA positive rate is high, but not 100%. Although PBC is usually slow, but its survival rate compared with the same gender and age groups is low. Clinically manifested as fatigue, pruritus, portal hypertension, osteoporosis, skin yellow warts, fat-soluble vitamin deficiency and recurrent asymptomatic urinary tract infections. In addition, they can still associated with non-hepatic autoimmune abnormalities. PBC in recent years, increasing the detection rate, but its pathogenesis is still completely clarified the end of the treatment is also a lack of specific control measures. PBC has recently introduced treatment AASLD guidelines, designed to help physicians understand the PBC diagnosis, assessment of severity and associated with other abnormalities, and may be useful for some patients treatment, prevention recommendations.
A, PBC diagnosis
Many cases of PBC in the regular examination revealed serum anti-mitochondrial antibody (AMA) positive, without any symptoms and abnormal liver function. Serum AMA testing course also the possibility of false positive results.
1. Biochemical examination: liver-derived serum alkaline phosphatase (ALP) elevation is the most common biochemical abnormalities PBC. Despite the small number of patients at diagnosis based direct bilirubin bilirubin increased, but increased hyperbilirubinemia late for the performance of PBC, and is a good indicator to determine the prognosis of PBC. In any chronic cholestatic liver disease, serum total cholesterol may be increased.
2. Bile duct imaging: for all patients with cholestatic hepatobiliary system should be carried out B-ultrasound. B-bile duct system of normal and AMA suggest positive patients do not need to be diagnosed cholangiopancreatography PBC. The diagnosis is not clear if the PBC or the sudden increase in serum bilirubin, the bile duct imaging is necessary.
3. Anti-mitochondrial antibody (AMA): AMA-positive serum is an important immunological features of PBC. The simplest and most cost effective method is to use rat stomach and kidney were determined by immunofluorescence. In PBC patients, AMA is generally present as a high titer (> 1:40), while low titer (<1:40) AMA-positive diagnosis of PBC is not specific, can be measured in the complex to negative. Determination of AMA for the most specific substrate for the 2 - oxidoreductase, it can be more specific immune spot technology to detect cases of AMA-positive PBC its specificity and sensitivity of detection of more than 95%. About 1 / 3 of the PBC patients antinuclear antibody and anti-smooth muscle antibodies.
4. Immune globulin: PBC patients with elevated immunoglobulin IgM-based, IgA is usually normal, but IgA deficiency reported. Ig generally used only for PBC detection of suspected cases. In AMA-negative patients, the increase of the IgG immunoglobulin-based, and IgM increase is not common.
5. Liver biopsy: the PBC AMA-positive and has a typical clinical presentation and biochemical abnormalities in patients with liver biopsy in the diagnosis is not required. PBC histologically divided into four phases: of the portal area of granulomatous inflammation associated with bile duct damage; period of bile duct hyperplasia accompanied by portal inflammation around; of spacing and bridging necrosis fibrous form; of the liver hardening period. Liver fibrosis and cirrhosis of the liver biopsy see a poor prognosis. The performance of the PBC histological damage to the main bile duct, it must have a sufficient number of samples of portal area organizations. Although the PBC histologically divided into four clear, but in a biopsy specimen, it can be both typical of the performance of different periods. However, no histological examination for the diagnosis of cirrhosis of the liver of PBC patients is not specific.
6. AMA-positive and normal liver function PBC: there is a group of scholars and ALP normal and asymptomatic AMA-positive patients, 29 patients were followed up for liver biopsy tissue and found 12 patients with liver histological diagnosis, only 2 patients with normal liver histology. 24 patients followed up for 10 years found that the AMA is still positive and significant in all cases evidence of cholestasis, of which 22 patients had clinical symptoms.
7. AMA negative PBC: Several scholars have reported that some PBC patients with typical clinical symptoms, biochemical and histological features of all the performance, but the AMA continued to negative. These patients are often described as "autoimmune cholangitis" or "self-autoimmune cholangitis." In addition to non-organ specific antibodies (high titers of anti-nuclear antibody and / or anti-smooth muscle antibodies), and similar to autoimmune hepatitis, its very likely is the subtype of PBC.
8. AMA positive and AMA-negative PBC and the natural history of patients with the autoimmune condition are very similar. However, AMA-negative PBC in ways similar to autoantibodies in autoimmune hepatitis, the combination of histology and liver function careful differential diagnosis is essential. With a histological diagnosis of PBC typical performance of 200 patients were reed and found, of which 12% of cases without positive autoantibody markers.
Second, the proposal on the diagnosis of PBC
1. If the patient has unexplained elevated ALP (ultrasound showed normal bile duct), the AMA has great value check.
2. AMA positive with biochemical cholestasis on performance, and no other reasons can explain, they can make the diagnosis of PBC, liver biopsy histology tend to confirm the diagnosis.
3. AMA-positive patients with normal ALP and should be followed up and liver function tests every year.
4. Patients with no other reason can be explained by increased ALP (normal bile ducts on ultrasound) and AMA-negative, then the determination should be immune globulin, or even liver biopsy histology.
If the AMA-negative or low titer AMA show weak positive or patients with elevated aminotransferases based biochemical, the liver biopsy for definite diagnosis of PBC or exclude the diagnosis of PBC is required. There are many other causes can lead to chronic cholestasis, intrahepatic bile duct can lead to disappearance of the majority. To late stage cirrhosis, histological features alone is difficult to make PBC diagnosis.
Third, PBC specific treatment
All patients with abnormal liver function should be carried out specific treatment. Application of immunosuppressive therapy has not yet extended the life of PBC patients reported that ursodeoxycholic acid (UDCA) although not reduce the demand for liver transplantation in patients, but can be an overall improvement in serum biochemical parameters of cholestasis, delaying the need for liver transplant patients time, and may prolong life.
1. Ursodeoxycholic acid damage caused by hydrophobic bile acid retention in the liver cells may be the main reason for the progress of PBC lesions. UDCA can promote the PBC, primary sclerosing cholangitis patients with intrahepatic bile from the liver into the bile duct cells, thereby reducing the intracellular levels of hydrophobic bile acids, play a role in protecting the cell membrane. UDCA also has also immunomodulatory effects. UDCA therapy can significantly improve biochemical markers of cholestasis patients, delaying the occurrence of portal hypertension in patients and reduce the incidence of esophageal varices, but it did not reduce the probability of variceal bleeding, and fatigue, itching of the effects are not sure Osteoporosis is invalid. Even if the long-term use PBC UDCA can not delay the progression of the disease still needs to end the sexual development of liver transplantation. Amount of UDCA day or one-time Dayton clothing graded the same effect, but the latter seems a better clinical compliance. UDCA rare side effects, mainly diarrhea. There are several joint a small sample of ammonia chatter pyridine UDCA, colchicine alkaline, or information prednisone PBC and found that the effect of combination therapy is not better than UDCA alone. Recommendations: PBC progress extremely variable, of asymptomatic patients in the study showed that the survival rate compared with the same surname other people the same age is lower. Asymptomatic patients, and UDCA treatment response is not sure. UDCA on liver function of patients return to normal liver function than those who never change the effect. So the best choice for liver dysfunction associated with PBC, UDCA treatment of patients with application of the dose 13 ~ 15mg/kg/d, or one ton service points. If both the application of cholestyramine, the two should be an interval of 4 hours or more.
2. Immunosuppressive therapy because PBC is an autoimmune disease, there have been several randomized controlled trial to study the efficacy of immunosuppressive drugs.
3. PBC liver transplantation is an indication for liver transplantation. Although some data suggest that recurrence after liver transplantation for PBC, but the recurrence rate is very low, and slow disease progression. It is recommended for end-stage PBC underwent liver transplantation is reasonable. The most reliable indicator of prognosis in PBC, serum bilirubin level and Mayo risk score. Mayo risk score: R = 0.872 e [bilirubin (mg / dl)] -2.53 e [albumin (g / dl)] 0.039 age (years) 2.38 e [prothrombin time (seconds) ] 0.859 edema score (0,0.5,1 points). However, in some cases, elevated bilirubin PBC lesions does not necessarily lead to deterioration, such as Gilber's syndrome, sepsis, pregnancy / hormone replacement therapy / oral contraceptives, bile duct stones, untreated thyroid disease, hemolysis, and toxic liver injury. In addition, there have recently been reported that the bilirubin level and Mayo risk scores for patients treated with UDCA has been applied there is no value, and UDCA treatment of PBC after liver transplantation can not change the results. Recommendation: for PBC patients progress to liver failure, or uncontrollable itching, severe osteoporosis, liver transplantation surgery recommended.
Fourth, PBC complications
Symptoms and associated complications for the PBC (such as malabsorption / portal hypertension and / or osteoporosis) treatment is essential.
1. Skin itching pruritus currently no effective treatment for the classic. Oral anion exchange resins cholestyramine for the treatment of pruritus in the first-line drugs. If the patient is not uncomfortable side effects of cholestyramine, rifampicin can be used as second-line drugs. Rifampicin can be well controlled PBC, itching, but it is not effective for all patients. The effect of treatment often significantly after 1 month. Rifampicin may bile acid in liver cells by altering the internal environment and to improve biochemical markers of PBC patients, to only Shatter effect. Nalmephene, Naltrexone and other opiates can be used to cholestyramine and rifampicin invalid patients. There are many other methods (such as UV, light and plasma rid therapy, etc.) for the PBC to control itching, but did not through formal clinical trials to be confirmed. Can not control the persistence of pruritus can be liver transplant.
2. PBC clear diagnosis of osteoporosis after bone mineral density should be monitored regularly, later followed up once every 2 years. Patient education to develop good habits (such as normal daily routine, quitting smoking), and vitamin D and calcium. Recommended postmenopausal female patients of hormone replacement therapy, and is best administered through the skin. Obviously if osteoporosis can be applied biophosphonate treatment.
About 11% of PBC patients have osteoporosis. Calcium and vitamin D to prevent decalcification, the recommended dose were 1 000 ~ 1 200mg / d and 25 000 ~ 50 000 IU, 2 or 3 times a week. Recent study found that raloxifene prevents bone loss and reduce serum cholesterol. 60 mg / d, continuously taking 1 year, can increase quality of lumbar spine bone mineral density (LS2BMD), no liver toxicity. In addition, Allen can effectively increase the bone mass of phosphate
3. SICCA syndrome of PBC patients should be asked of all dry eye, dry mouth and difficulty swallowing, with or without symptoms, patients should ask women whether sexual difficulties, if any, should be given appropriate treatment.
4. Reynolds syndrome patients in the cold regions, Renault syndrome treatment is a difficult problem, patients should avoid exposure to cold hands and feet, the environment, smokers should quit. Can be applied when necessary, calcium antagonists, but may increase lower esophageal sphincter dysfunction.
5. Portal hypertension in cirrhotic PBC patients to be prior to the development of preantral of portal hypertension, cirrhosis of the liver treatment of portal hypertension patients with other types of cirrhosis. However, -blockers for non-cirrhotic portal hypertension before sinus efficacy yet to be confirmed, if necessary, consider shunt surgery. PBC for the first time explicitly recommended that diagnosis should be screened with or without the presence of esophageal varices, followed by a 2-year re. If identified, varicose veins, which should take measures to prevent bleeding.
6. Fat-soluble vitamin deficiency can be complicated by hyperbilirubinemia fat-soluble vitamin deficiency and calcium malabsorption, in the absence of jaundice patients, their fat-soluble vitamin levels and little is known about the value of oral supplementation. Of the best fat-soluble vitamin supplement to give water-soluble form. Monthly subcutaneous injection of vitamin K can correct secondary to a lack of vitamin K due to the coagulopathy.
7. Thyroid disease thyroid disease can affect about 15% to 25% of PBC patients, which usually can be there before the onset of PBC patients. Recommended that patients diagnosed with PBC should be measured in serum thyroid hormone levels, and regular inspections.
8. Pregnancy, pregnancy problems on the PBC patients rarely reported. In most cases, pregnancy can lead to itching or pruritus patients had increased, mainly caused by high levels of estrogen cholestasis role. There were also reports of cholestasis prompted higher miscarriage. For the performance of PBC patients with cholestasis of pregnancy outcomes has not yet good evidence. Recommended as treatment for PBC in all of the safety of the first three months of pregnancy is not clear, so the first three months of pregnancy, all of the treatment should be discontinued. UDCA in the three months of pregnancy is safe, and improve maternal cholestatic symptoms effective. Pregnant women to determine whether endoscopy should be the presence of varicose veins and, if selected should be given Buffet receptor resistance agent. Obstetricians should recommend the patient to minimize the labor intensity of mid-pregnancy.The disease is unexplained, chronic progressive cholestatic liver disease, may be autoimmune related.The disease often associated with other autoimmune diseases such as rheumatoid joint class, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis and other co-exist, more common in middle-aged women, insidious onset, after a slow, early symptoms of mild Patients generally in good condition, no significant loss of appetite and weight declined more than about 10% of patients may have no symptoms. Of unexplained chronic progressive obstructive jaundice patients, especially those associated with steatorrhea, the incentive should learn more about the onset and progression of the situation, whether the existence of other autoimmune diseases, attention and secondary biliary cirrhosis cause jaundice and other liver cirrhosis were identified.
Experience Findings
Skin, sclera jaundice shows multiple scratches and scaling. Liver, splenomegaly surface is still smooth, no tenderness.
Symptoms
Primary biliary cirrhosis and other autoimmune diseases, such as regular rheumatoid joint class, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis and other co-exist, more common in middle-aged women, insidious onset, after slow Early symptoms are mild, the patient generally in good condition, no appetite and body weight decreased significantly more, about 10% of patients may have no symptoms. Of unexplained chronic progressive obstructive jaundice patients, especially those associated with steatorrhea, the incentive should learn more about the onset and progression of the situation, whether the existence of other autoimmune diseases, attention and secondary biliary cirrhosis cause jaundice and other liver cirrhosis were identified.
Signs
Skin, sclera jaundice shows multiple scratches and scaling. Liver, splenomegaly surface is still smooth, no tenderness.
Auxiliary examination
Lipids, serum bile acids, conjugated bilirubin, AKP, and GGT enzymes and other micro-duct was significantly increased, transfer of nitrogen enzymes normal or mild to moderate increase. Anti-mitochondrial antibody-positive blood, IgM increased, prothrombin time prolonged. Positive for urinary bilirubin, urine bile per normal or reduced.
Imaging ultrasound, ERCP, CT, PTC, etc. to understand whether the extrahepatic bile duct dilatation and obstructive jaundice caused by extrahepatic disease.
Treatment
Proper rest, give high-protein, high carbohydrate, low-fat diet high in vitamins, daily fat
Prevention of common sense:
Primary biliary cirrhosis is an inflammation of the intrahepatic bile duct damage, characterized by intrahepatic cholestasis with chronic liver disease, often accompanied by a variety of immunological abnormalities, the incidence may be related to immune abnormalities. Typical clinical manifestations are, diagnosis, and more without difficulty, but this time the disease is more advanced, so early diagnosis is to determine the treatment success and failure. With the launching of sensitive detection methods, asymptomatic, primary biliary cirrhosis without jaundice gradually increasing the early diagnosis, diagnostic value of early detection methods are ALP and anti-mitochondrial antibodies. Confirmed mainly by pathological examination in the diagnosis of primary biliary cirrhosis, should be a variety of causes obstructive jaundice, viral hepatitis, bile capillary, drug-induced jaundice, primary sclerosing cholangitis, etc. differentiated disease. There is currently no effective treatment, the treatment of primary biliary cirrhosis is the main purpose of: (1) prevention and treatment of complications of intractable cholestasis; (2) dealing with the various manifestations of decompensated liver cirrhosis; (3) If possible, the implementation of the treatment on the disease process, but should pay attention to side effects. Associated with liver decompensation and (or) the poor quality of life of patients can be considered for liver transplantation.
Prevention of primary biliary cirrhosis
The disease of unknown etiology, chronic progressive cholestatic liver disease, may also be related to autoimmune drug reaction, infection and other factors related to the prevention of the disease the following way of life help:
1, absolute alcohol (including beer and wine), cutting a variety of drinks, to drink hot tea.
2, constant daily diet to low salt, low fat, low sugar, high protein is good, do not eat spicy, greasy, fried, sticky hard food, do not eat too much, and pay attention to food hygiene, prevention of diarrhea .
3, try to eat foods are harmful to the liver.
4, maintaining regular exercise and a good attitude.Primary biliary cirrhosis (Primary biliary Cirrhosis, PBC) is a chronic progressive disease, mainly for the destruction of intrahepatic bile duct with portal inflammatory changes, eventually leading to fibrosis and cirrhosis. Foreign reports about the prevalence of PBC 2-24/10 million, the annual incidence rate is about 0.4-3/10 million [1]. PBC disease at any age can be seen mainly in middle-aged women (M: F = 6-16:1), but rare in children. Have a family tendency, a relative prevalence of 4282/10 million, and the normal population of only 7.5/10 million, but on a population-based case control study showed that PBC, PBC's family, not as previously reported tendency as significant, but not with smoking history, history of psoriasis and eczema was related to [2].
One. Clinical manifestations.
More than half of PBC patients have skin itching, may be associated with fatigue, sleepiness. There were other symptoms: hyperpigmentation, xanthoma, malnutrition, diarrhea, osteoporosis and symptoms of hepatic decompensation: jaundice, ascites, varices, and bleeding. Intrahepatic lesions than non-drinking patients with long-term, enabling early detection of hepatosplenomegaly PBC.
1. Clinical stage: in four.
I, liver function normal, asymptomatic stage, can be detected AMA-positive. 76% of the PBC in patients with clinical symptoms appear within 2 years, 83% of abnormal liver function occurred within 5 years.
II period, abnormal liver function period: 5 years 80% of patients with symptoms
III, duration of symptoms, mainly for skin itching,
IV, decompensated, mainly for the performance of liver decompensation: jaundice, ascites, upper gastrointestinal bleeding, diagnosis to death an average of 3-5 years.
2. Concomitant diseases: PBC patients often accompanied by other autoimmune diseases, nearly 30% associated with Sjogren's syndrome. 25% associated with autoimmune thyroiditis. Is released with other common, such as joint pain, Raynaud's phenomenon, fingertip hardening and fiber alveolar go far. There are a small number of patients associated with systemic lupus erythematosus, thrombocytopenic purpura and so on.
II. Laboratory tests.
Abnormal liver function with cholestasis, performance-based, serum AKP, r-GT, 5'-NT increased, but normal or mildly elevated transaminases and rarely more than 5 times. Late bilirubin, and parallel with liver failure. Often elevated serum cholesterol levels, high density lipoprotein levels increased early, then gradually declined. Because cholestasis, malabsorption of fat-soluble VitK, can cause prolonged PT. Elevated serum immunoglobulin, the IgM was significantly higher, 96% of patients with AMA, AMA has many subtypes, there are many diseases such as drug damage, heart disease and so may appear, the E2 isoform is the PBC-specific antibodies. But can also be a variety of antibodies, such as ANA, anti-SSA, anti-SSB antibodies, anti-centromere antibodies. About 25% PBC patients there is also anti-nuclear antibodies PBC specific antibodies. 50% AMA-negative PBC existence of such antibodies. In still another 1% PBC specific anti-nuclear pore protein complex antibodies.
III. Pathologic features.
PBC lesions of early portal area, see the bile duct as the center of lymphocytes, plasma cells and eosinophil infiltration, recently that the portal area eosinophils and peripheral areas eosinophils increase is the PBC early common and characteristic manifestations [ 4.5]. Bile duct epithelial cells of pleomorphic epithelioid cell damage and granuloma formation. With the increase of inflammatory necrosis, portal area and bile duct damage in the full absence of reaction around the bile duct hyperplasia, breaking the limiting plate immersed in hepatic parenchyma, known as "bile duct necrosis of debris." Late lesions, fibroblast activation, extensive fibrosis in portal area, the final formation of extensive fibrous septa and regenerative nodules, cirrhosis of the liver formation.
Staging: There are two ways, Scheuer staging and Ludwing stage, both of which will be divided into four PBC. The main difference is in the Scheuer stage I, II of the distinguishing characteristic is that in the bile duct damage (flower blossoms like bile duct damage) based on the availability of bile duct hyperplasia. The Ludwing staging I, II of the distinguishing point of emphasis is necrotizing inflammation confined to portal area, which is also the past, chronic persistent hepatitis and chronic active hepatitis of defined points. Two phases III, IV line similar to that in the basis of the formation of fibrous septa with or without cirrhosis (Table 1).
Liver biopsy in the diagnosis or prognosis of PBC is not necessary. AMAE2 positive patients tend to have the typical pathological features of PBC, so when there is AMAE2 positive and PBC patients clinical manifestations, can be diagnosed as PBC, pathology of cirrhosis without too much significance on the prognosis, because patients with PBC can occur early in the pathological sinus before the clinical manifestations of portal hypertension. However, serum and other clinical manifestations and diagnosis of atypical and when the selection and application of certain drugs and efficacy evaluation of other aspects of their value.
IV. Pathogenesis.
There is evidence that the disease cellular and humoral immunity are abnormal.
Cellular immune abnormalities mainly as follows:
1. Biliary epithelial cells in most patients with abnormal surface expression of HLA;
2. Bile duct inflammation around the damaged area and portal area there are lymphocytes, CD4 and genotyping are CD8;
3. Lymphocyte subsets in peripheral blood changes, appears self-reactive T cells (autoreactive T cell), concanavalin agglutinin inhibition was caused by abnormal changes;
4. The specific T cell receptor in patients with no apparent selectivity, but the cell adhesion molecule (ICAM-1) in many epithelial cells, especially lymphocytes damaged area was highly expressed. These molecules may act as auxiliary factors that promote destructive lymphocyte interactions with target cells.
Humoral immune abnormalities mainly for anti-mitochondrial antibodies and related antigens. More than 90% of patients with primary biliary cirrhosis anti-mitochondrial antibodies. Currently known, the antigen recognized by such antibodies are mainly distributed in some mitochondrial enzyme complex E2 component (E2component), the enzyme complex, including malate dehydrogenase complex (PDC), branched-chain keto acid de- hydrogenase complexes and oxalyl glutaric acid dehydrogenase complex. Patients with primary biliary cirrhosis in anti-mitochondrial antibodies directed against the major antigen is PDCE Now that the primary biliary cirrhosis, bile duct abnormal expression of HLA resembles the surface of epithelial cells epitopes E2 component, so that these T cells in biliary epithelial cells become the target of attacks, causing bile duct injury.
Five. Diagnosis.
= 1:40); B ultrasonic examination no signs of extrahepatic bile duct obstruction, to diagnose the disease, if the AMA negative, serum immunoglobulins and should be done to help diagnose liver biopsy. If only the AMA-positive patients, but the AKP is not high, should be regularly followed up.Primary biliary cirrhosis (Primary Biliary Cirrhosis, PBC) is a small intrahepatic bile ducts mainly non-suppurative is characterized by progressive damage of autoimmune diseases. The clinical manifestations of PBC was recognized 150 years of history, but only from 1965 that the disease specific markers - anti-mitochondrial antibody (AMA), the clinic began extensively diagnosis of PBC; if purely for diagnostic purposes is not in favor of each patient of liver histology. AMA titer correlated with the severity of the disease are not related, AMA in PBC pathogenesis is unclear. Research shows that the incidence of social family: AMA-positive women are at high risk of PBC, genetic factors and environmental factors are key factors in the pathogenesis of PBC. PBC features of liver injury can be expressed in normal bile duct epithelial cells of the abnormal immune response can also be manifested as abnormal bile duct epithelial cells of normal immune response. PBC characteristic features just for the interlobular bile duct damage and bile duct spacing, it does not affect the large bile ducts, so no clinical imaging findings of biliary obstruction, the same epithelial damage may also occur in salivary glands and epithelial cells of the pancreas, called the clinical appear "syndrome" symptoms.
European and American epidemiological survey found that women over 40 years the incidence of PBC is about 1 / 600. In the past 20 years, its incidence has significantly increased trend, perhaps because of the early cases were patients with very early detection and life expectancy. PBC in liver cirrhosis mortality accounted for 0.6% to 2%. The incidence of PBC significant regional differences in northern England and North America, the highest incidence. Past that the disease is rare in China in recent years as people deepen the understanding of this disease and found that PBC patients are not uncommon in China, reof domestic literature in 2000 just before the case of PBC cases reported in 2001, five cases of summary reports The total number of cases 87 cases reported up to the year 2004 more than 20, the total number of cases over 2000 cases. In 2005 reported more than 40 articles, the number of cases reported has more than 5000 cases or more. Our hospital from 2001 to carry out regular anti-mitochondrial antibody test, the weekly average 1-2 new cases found in PBC patients has found that more than 300 cases of PBC patients. We had 5,000 healthy individuals with anti-mitochondrial antibody test was found positive in 8 cases, after further confirmation, there are 6 cases of PBC. Lack of specific clinical manifestations of PBC, it can easily be misdiagnosed. Some units reported that the initial misdiagnosis rate of 60 ~ 90%, most misdiagnosed as viral hepatitis (not type) or non A - non-E hepatitis, but also misdiagnosed as drug-induced hepatitis, fatty liver, cholelithiasis, etc.; and Most of the time of misdiagnosis are longer, up to several years or even ten years or more. Most of the misdiagnosed cases were not appropriate treatment, some treatment increased liver damage, have also accelerated the progress of the disease.
PBC is immune-mediated disease, but immune therapy is not ideal. PBC patients and in experimental treatment were very difficult to accomplish, since most cases are early and no symptoms. Is also not found a good surrogate marker for long-term illness followed up. If the progression to liver failure, liver transplantation is the only effective method. However, any research that is a "fuzzy" end. Ursodeoxycholic acid (UDCA) in patients although not completely cured, but can slow down the progress of fibrosis, reduce cholestasis. PBC, treatment also includes the control and prevention of disease caused by liver and extrahepatic complications. Only when we truly understand the pathogenesis of the disease, will have hoped to find a specific targeted therapy.
A, PBC's History
Are the first documented description of PBC may be associated with the same disease, now is the of Addison and Gull, 1851 in Guys Hospital years they yellow one case of skin tumors were reported when patients with the disease [1]. 1875 Hanot recognize cholestasis is the essence of the disease, so after some scholars call it Hanot's xanthomatous cirrhosis or liver cirrhosis, cholestatic liver disease because the yellow tumors on the skin [2]. The name of primary biliary cirrhosis was established in 1949, when the purpose is to boldly and extrahepatic duct obstruction secondary to cirrhosis of the liver caused by the phase difference [3]. 1950 Ahrens et al [4] a detailed description of primary biliary cirrhosis clinical manifestations, was that this is a rare disease, reported worldwide before 1950, no more than 100 cases. Initially named as a cirrhosis of the liver, so the name is often misunderstood, many patients are diagnosed when the PBC is not actually true cirrhosis, patients with this name will cause psychological discomfort. Some scholars try to use various other names, such as chronic non-suppurative granulomatous diseases such as intrahepatic bile duct, the end has not been universally accepted. Therefore, PBC is still used as the name of this disease are still in use.
Second, PBC describes the initial clinical manifestations
1959 Sheila Sherlock describes her personal follow-up from 1944 to 1959 of the 42 cases PBC [5], 20 patients presented with skin itching, or even significant jaundice in 11 years. However, there were 14 cases of jaundice occurred after the itching. There are several cases of liver complaints but no symptoms of a large (possibly the first description of asymptomatic PBC). At the time, no effective way to distinguish intrahepatic and extrahepatic bile duct obstruction, the group of patients no patient had symptoms of fever or abdominal pain. Stool color change more, the majority of normal, small white stool. Since there was no ultrasound, so often out to establish the diagnosis of bile duct exploration after scientific examination or liver biopsy determined. 42 patients, only 5 cases have not been confirmed after exploratory surgery.
Sherlock xanthoma cases reported skin changes are very common. 42 cases, 16 cases had xanthoma, xanthoma some flat, others are deposited in the eyelid nodular surface of the inner canthus, can also appear in the wrist, hip, knee and ankle, never occurs in the tendon sheath before. These patients are not all caused due to high cholesterol deposits in the skin caused by cholesterol. Patients by autopsy findings in 15 cases of vascular atherosclerosis in these cases are rare. She reported that the degree of hyperbilirubinemia and the percentage of dietary fat absorption correlation.2 Results


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