What is autoimmune liver disease?

  Autoimmune liver diseases are a group of diseases caused by the body’s immune system attacking its own liver tissues, and the common clinical autoimmune liver diseases include autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Primary biliary cirrhosis is the most common, followed by autoimmune hepatitis, while primary sclerosing cholangitis is less common than the first two. These three diseases have their own characteristics in terms of clinical manifestations and laboratory tests. Autoimmune hepatitis mainly manifests as inflammatory necrosis of hepatocytes and primary biliary cirrhosis, while primary sclerosing cholangitis mainly manifests as intrahepatic cholestasis, and the appearance of autoantibodies may play an important role in the process of autoimmune liver disease.  1, autoimmune hepatitis Autoimmune hepatitis is a kind of chronic hepatitis, more common in women, clinically may have weakness, loss of appetite, abdominal distension, menopause, etc.. There is a significant increase in ALT and autoantibodies are detected in the serum. AIH is divided into three types according to autoantibodies: ANA and/or SMA are detected in type I, LKM1 and/or LC1 antibodies are detected in type II, SLA/LP antibodies are detected in type III, and hyper-IgGemia is present, and liver tissue biopsy is needed to further clarify the diagnosis if necessary. The disease can have manifestations of various autoimmune diseases, such as arthropathy and thyroid disease. The main drugs for treatment are glucocorticoids and immunosuppression. The development of cirrhosis requires prevention of complications such as upper gastrointestinal bleeding, ascites and hepatic encephalopathy.  2.Primary biliary cirrhosis Primary biliary cirrhosis is most common in middle-aged and elderly women, with the ratio of men to women being 1:9. Most patients have an insidious onset and progress to cirrhosis by the time it is detected. Some patients have itchy skin as the first symptom, and later the symptoms gradually worsen. There may be jaundice, hepatomegaly, abdominal pain, subcutaneous fat deposits, abnormal darkening of the skin, light yellow spots on the eyelids, steatorrhea, and recurrent urinary tract infections. Serum aminotransferases are mildly to moderately elevated, immunoglobulin IgM is elevated, serum alkaline phosphatase (ALP), glutamyl transpeptidase (GGT), serum cholesterol and lipoproteins are elevated, positive anti-mitochondrial (AMA) antibodies and positive M2 are important features of the disease. The treatment is mainly ursodeoxycholic acid at a dose of 13-15 mg/kg/d, which can be given orally in 2-3 doses, and combined with other liver-protective treatments.  Primary sclerosing cholangitis Primary sclerosing cholangitis is a chronic cholestasis syndrome of unknown etiology, mostly in young and middle-aged males. 70% of cases are combined with inflammatory bowel disease (mainly ulcerative colitis). Typical symptoms are jaundice, itching, general malaise, restlessness or feeling sick, loss of appetite, dyspepsia, hepatomegaly and splenomegaly. Laboratory tests show mild to moderate elevation of serum transaminases, increased clear alkaline phosphatase (ALP), glutamyl transpeptidase (GGT), and may be positive for autoantibodies ANCA, and diagnosis requires endoscopic retrograde cholangiopancreatography (ERCP). Treatment is based on ursodeoxycholic acid at a dose of 13-15 mg/kg/d, which can be given orally in two to three doses, and endoscopic balloon dilatation can reduce symptoms.  The detection of autoantibodies in patients with liver disease does not necessarily mean that they have autoimmune liver disease. Autoantibodies can also be detected in viral hepatitis and drug-related hepatitis, but most of them have low autoantibody titers, so they need to be analyzed in the context of specific situations. With the promotion of testing technology and awareness of the disease, the number of reports of autoimmune liver disease has increased significantly, but early detection and early treatment will lead to better outcomes.