Clinical features and treatment of autoimmune liver disease

  Autoimmune liver diseases are a group of non-infectious chronic liver diseases, and their clinical symptoms do not differ clearly and specifically from those of general chronic viral hepatitis. Although the confirmation rate of autoimmune liver diseases has improved in recent years with the continuous progress and wide application of immunological detection techniques, there are still many cases with some difficulty in clinical diagnosis.  Autoimmune liver diseases mainly include primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH).  PBC is a cholestatic chronic liver disease characterized by progressive non-purulent inflammatory destruction of small intrahepatic bile ducts, which may eventually lead to cirrhosis and liver failure; the cause of PBC is unknown, and the detection rate of positive serum anti-mitochondrial antibody (AMA) is as high as 95%; treatment can be applied with ursodeoxycholic acid (UDCA) 13-15 mg/kg/d to reduce intrahepatic cholestasis.  PSC is a chronic cholestatic liver disease of unknown etiology, characterized by non-suppurative inflammation and scar formation in the intra- and extra-hepatic bile ducts, which can eventually progress to biliary cirrhosis and liver failure; the peak age of onset of PSC is 30-40 years old, but also seen in young children and elderly people, and is more common in men; there is no specific treatment for PSC, but mainly symptomatic treatment, with liver protection, enzyme reduction, yellowing, etc. The main treatment for PSC is symptomatic, with liver protection, enzyme lowering and yellowing.  AIH is an intermittently progressive chronic liver disease characterized by symptoms of hepatitis of varying severity, immunological abnormalities, prominent inflammatory lesions in the peripheral hepatocytes of the confluent area (interfacial hepatitis), and a good response to corticosteroid immunosuppressive therapy. The majority (70%) of the affected population is female, and the characteristic autoantibodies are positive for antinuclear antibodies (ANA) and anti-smooth muscle antibodies (SMA); treatment can be administered under physician supervision with immunosuppressive therapy along with hepatocyte membrane protection and enzyme lowering.  The three diseases of autoimmune liver disease may overlap. For the treatment of patients with overlapping syndromes, it is necessary to distinguish between hepatitis and cholestasis, and to give immunosuppressive treatment to those with significant inflammatory components of the liver and UDCA treatment to those with predominant cholestasis, and to consider combination therapy if necessary. Early diagnosis and early treatment of autoimmune liver disease is very important.