Patients often ask questions about autoimmune hepatitis online. In China, slow hepatitis B is the most common disease causing chronic liver damage, but in recent years, the incidence of autoimmune liver disease is on the rise, and autoimmune liver disease is usually divided into three types: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), in addition to those caused by the above There are also overlapping variant syndromes caused by the overlap and variation of several diseases, etc. The following is a description of the common AIH, so that patients suspected or diagnosed with this disease can have a preliminary understanding of the occurrence, development, treatment and prognosis of the disease, and better cooperate with the relevant examination and treatment to achieve better results. I. Overview: In 1950 Waldenstrom first discovered a specific type of chronic hepatitis, mainly seen in women, with jaundice, markedly elevated gammaglobulin and amenorrhea, which often develops into cirrhosis in later stages. Mckey later named it “lupoid hepatitis” because of certain similarities in clinical manifestations and autoantibodies to SLE. In 1992, the international conference referred to “autoimmune liver disease” and “autoimmune chronic active hepatitis” collectively as “autoimmune hepatitis”. In 1992, the international conference referred to autoimmune hepatitis (AIH) and autoimmune chronic active hepatitis as “autoimmune hepatitis”, and removed the restriction of 6 months or more, and identified the disease as a non-viral autoimmune disease. Definition: It is a disease of unknown etiology, characterized by hyperglobulinemia with multiple autoantibodies and chronic necrotizing inflammation of the liver. The basic pathological changes are debris necrosis around the liver lobules and also bridge-like necrosis with marked infiltration of lymphocytes, monocytes and plasma cells. Epidemiology: The prevalence of AIH is 0.1~1.2/100,000 in the population of Western Europe and North America. In Japan, it is about 0.015~0.08/100,000 people. It has not been reported in China. The cause is still unclear, there are several theories: autoimmune abnormalities (CTL, ADCC), genetic factors (HLA-DR3, HLA-DR4), viral infections (molecular mimicry theory), drug factors, food additives large consumption of packaged or processed potato chips, French fries, corn and wheat snacks, a variety of soft drinks, etc. IV. Typology: usually not for three types V. Common autoantibodies Anti-nuclear antibody (anti-nuclear antibody, ANA), anti-smooth muscle antibody (anti-smooth muscle antibody, SMA), anti-liver and kidney microsome antibody-1 (anti-LKM-1 antibody), anti-liver and kidney microsome antibody-1 (anti-LKM-1 antibody), anti-liver and kidney microsome antibody-1 (anti-LKM-1 antibody), anti-liver and kidney microsome antibody-1 (anti-LKM-1 antibody), anti-liver and kidney microsome antibody-1 (anti-LKM-1 antibody). anti-bodies to liver cytosolic protein type 1 (LC-1 antibody), anti-neutrophil cytoplasmic (anti-neutrophil cytoplasmic), anti-autoantibodies to liver-pancreas (LP), and anti-neutrophil cytoplasmic (anti-neutrophil cytoplasmic) antibodies. pancreas (LP) and anti-soluble liver antigen antibodies (SLA), anti-human asiaglycoprotein receptor antibodies (ASGPR antibodies) VI Pathological changes: Typical changes of chronic active hepatitis with marked infiltration of lymphocytes and plasma cells in the confluent area (interface hepatitis), with a small number of eosinophils and neutrophils. In general, debris necrosis (PN), bridging necrosis and panacinar necrosis are seen, and in severe cases, adjacent whole-alveolar necrosis fuses with each other to form submassive and massive necrosis. Steatosis is less common. The transition from highly active lesions to cirrhosis often occurs 2 years after the onset of the disease. Finally, it develops into large nodular cirrhosis. Clinical manifestations: 1. Onset and course of the disease: It often has a chronic and prolonged course. Most patients have a slow onset, and as the disease progresses, cirrhosis and portal hypertension may appear in the late stages. There are no specific symptoms at the onset of the disease, so it is easy to misdiagnose the disease as other diseases, and the disease is diagnosed only after the appearance of persistent jaundice and the detection of liver function and serum autoantibodies. Some patients may also have an acute onset, and about 25% of patients have an onset similar to that of acute viral hepatitis. 2, gender and age: Most commonly seen in women, the ratio of men to women is 1:4-6. The disease is most often seen in adolescents, with about 50% of patients between the ages of 10 and 20. Some patients develop in menopausal women. The main symptoms and signs: The patient’s symptoms are similar to those of chronic hepatitis, with common symptoms such as weakness, loss of appetite, nausea, aversion to greasy food and abdominal distension. Sometimes there may be low fever, pain in the epigastric or liver area. Irregular menstruation or amenorrhea is more common in female patients. Jaundice is more common, mostly mild or moderate, and profound jaundice is less common. About 20% of patients can have no jaundice. It may be accompanied by hepatosplenomegaly, spider nevus and liver palms. In progression to cirrhosis, ascites and swelling of the lower extremities may also occur. Extrahepatic manifestations: joint pain: symmetrical, wandering, recurrent, without joint deformity; skin lesions: rash, subcutaneous hemorrhagic spots or petechiae, capillaritis; hematological changes: mild anemia, leukocytopenia and thrombocytopenia, rare hemolytic anemia with positive Coombs test, a few may be accompanied by eosinophilia; chest lesions: pleurisy, pulmonary atelectasis, pulmonary interstitial fibrosis or fibroalveolitis Pulmonary arteriovenous fistula, pulmonary hypertension; renal lesions: glomerulonephritis, renal tubular acidosis. Endocrine disorders: similar to Cushing’s sign, Hashimoto’s thyroiditis, mucinous edema, hyperthyroidism, diabetes mellitus, breast enlargement in men, menstrual irregularities in women. it is not uncommon for patients with AIH to have rheumatic diseases such as SS, SLE, RA, etc. Some patients may have ulcerative colitis. 4, laboratory tests: liver function tests: serum bilirubin is often mildly or moderately elevated, with elevated serum transaminases, γ-glutamyl transpeptidase (GGT) and alkaline phosphatase (ALP) also elevated. γ-globulin is significantly elevated, which is one of the characteristics of AIH. Immunoserological examination: A variety of autoantibodies can be measured in the serum of AIH patients, which is the characteristic clinical manifestation of the disease and the main basis for diagnosis. 5. The following characteristics are summarized: Mostly seen in women. The onset of the disease is slow and insidious in most patients. Serum gamma-globulin level is significantly increased, mainly IgG. Serum transaminases are mildly or moderately elevated. Autoantibodies such as ANA, SMA, LKM, SLA/LP with high titers can be detected in the serum. Markers of viral hepatitis are negative. Histopathological examination of the liver shows histological changes of a slow-acting liver, such as debris-like necrosis in the confluent region or bridge-like necrosis between the central and confluent regions of the lobules, with marked lymphocytic and plasma cell infiltration. No bile duct injury. Exclusion of other causes of liver disease, such as viral hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, drug damage to the liver, hepatomegaly (Wilson’s disease), alcoholic liver disease, other autoimmune diseases, etc. No alcohol abuse and no recent use of hepatotoxic drugs. Effective for treatment with adrenocorticosteroids or immunosuppressive drugs VIII. Treatment The aim of treatment is to provide rapid remission and to keep the patient in a sustained remission period. 1. General treatment: appropriate restriction of physical activity and rest. Avoid alcohol. Eat a low-fat, high-protein and vitamin-rich diet. Avoid the use of drugs that are damaging to the liver. 2. Drug therapy: 2.1 Adrenocorticotropic hormone: The commonly used preparation is prednisone or prednisolone. Most patients treated with adrenocorticotropic hormone will have varying degrees of improvement in the pathological histology of the liver, in addition to a reduction in clinical symptoms and improvement in the laboratory indexes of liver function, although the results of follow-up observations by most scholars show that the chance of eventually developing cirrhosis is not significantly reduced. The commonly used dose is 40-60 mg of oral prednisone or prednisolone per day, and the course of treatment should be long. After the clinical symptoms and biochemical indexes of liver function improve and the disease obtains remission, the dose can be reduced, but the reduction must be slow; premature reduction or discontinuation of the drug will easily aggravate the disease again or relapse. If adrenocorticotropic hormone treatment alone does not bring about remission, combination therapy with immunosuppressive agents such as azathioprine can be considered. Prednisolone is the best choice for adrenocorticosteroids because after oral administration of prednisone, it must be converted to prednisolone in the liver before it can play a therapeutic role, and this conversion may be impaired when patients with impaired liver function. The side effects of adrenocorticotropic hormone include full moon face, acne, hirsutism, osteoporosis, weight gain, increased blood pressure, induction of diabetes, and susceptibility to secondary infections. 2.2 Azathioprine: The efficacy of azathioprine alone in the treatment of AIH is poor. It is usually used in combination with azathioprine in adrenocorticotropic hormone therapy because of unsatisfactory efficacy, large adverse reactions to adrenocorticotropic hormone, or when the disease has gone into remission after adrenocorticotropic hormone therapy. The common dose is 30-40 mg of prednisolone and 75-100 mg of azathioprine daily. azathioprine’s side effects are mainly inhibition of bone marrow proliferation, which must be taken seriously in high doses and long courses of treatment, and changes in blood picture should be observed. In addition, side effects such as mucosal ulcers, nausea, loss of appetite and hair loss can also occur. 2.3 Other hepatoprotective drugs: such as glycyrrhetinic acid preparations, silymarin preparations, various types of Chinese herbal medicines with hepatoprotective effects, ursodeoxycholic acid, etc. 3.1 Monotherapy: children, young women who are pregnant or intend to become pregnant; patients with malignancy; patients with marked leukopenia; patients who have lost tolerance to azathioprine (e.g., azathioprine methyltransferase deficiency) 3.2 Combination therapy: adults with AIH and no contraindications to azathioprine, e.g., leukopenia, drug-related biliousness; postmenopausal women, obese, emotionally unstable; diabetic, unstable high blood pressure, etc. Postmenopausal women, obese, emotionally unstable; diabetes mellitus, unstable hypertension, osteoporosis, acne, etc. Monotherapy Combination therapy Week 1 Prednisone 60 mg/d Prednisone 30 mg/d, azathioprine 50 mg/d Week 2 Prednisone 40 mg/d Prednisone 20 mg/d, azathioprine 50 mg/d Week 3 Prednisone 30 mg/d Prednisone 15 mg/d, azathioprine 50 mg/d Week 4 Prednisone 30 mg/d Prednisone 15 mg/d, azathioprine 50 mg/d Maintenance Prednisone 20 mg/d Prednisone 10 mg/d, azathioprine 50 mg/d 3.3 Criteria for determining the efficacy of immunosuppression: Complete remission ①Symptoms improve significantly or basically disappear. ②Liver function improves. ③Hepatic biopsy returns to normal, or shows minimal lesion activity. Partial remission The liver function does not return to normal by 12 months of treatment; or liver function returns to normal but liver histology still shows persistent inflammation. Ineffective Regardless of the improvement of symptoms, one of the following is considered ineffective: ① No further improvement by 6 months of treatment. (ii) No significant improvement in liver biopsy before or after treatment. Failure of treatment Even though indicators suggestive of disease activity have improved, the symptoms have worsened and the disease has deteriorated. Relapse After complete remission with treatment, ALT/AST rises again to more than 2 times the upper limit of normal, and liver biopsy shows reactivation of the lesion. Liver transplantation Patients who have not been treated with medication and have advanced disease can be considered for liver transplantation. After liver transplantation, AIH can still recur, so immunosuppressive therapy must be continued to reduce the recurrence rate of AIH. There is no clear relationship between persistent positive autoantibodies and AIH recurrence. Patients who relapse after discontinuation of the drug can still resume the induction regimen started and complete remission can usually still be induced again. Given the high incidence of adverse reactions to immunosuppression in patients with multiple relapses, after remission is achieved with induction therapy (disappearance of clinical symptoms and return of ALT/AST to less than 2 times the upper limit of normal), maintenance therapy with low doses of immunosuppressive agents should be applied for long-term use to maintain remission. The response of AIH patients to immunosuppressive therapy is known to be related to their genetic background, with fewer complete remissions and more relapses in HLA-DR3-positive patients and the opposite in negative patients. X. Prognosis The prognosis of autoimmune hepatitis is related to the severity of inflammatory activity and the genetic quality of the host, with a 10-year mortality rate of 90% in severely ill patients without treatment. With appropriate immunosuppressive therapy, approximately 60% of patients with highly active autoimmune chronic liver disease have quiescent lesions within 3 years, with an average 5-year survival rate of 90%.