What Is an Autoimmune Liver Disease?

Autoimmune liver disease (ALD) is a special type of chronic liver disease caused by immune dysfunction in the body, including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and the so-called overlap syndrome that overlaps each other. Different types of autoimmune liver diseases have different demographic characteristics, clinical manifestations, and pathological changes of the liver. The specific pathogenesis of the disease is unknown, and patients are often accompanied by other autoimmune diseases such as diabetes and Hashimoto's thyroiditis.

Basic Information

Visiting department

Gastroenterology

Common locations

liver

Common causes

The etiology is unknown and is related to the body's immune dysfunction.

Common symptoms

Weakness, decreased appetite, bloating, discomfort in the liver area, yellowing of the skin and sclera.

Causes of autoimmune liver disease

The etiology of this group of diseases is not completely clear, and may be related to various factors, such as infection, physical or chemical factors, and immune dysfunction. The clear precipitating factors are various hepadnavirus infections.

Viral infections (such as hepatitis B virus, hepatitis C virus, EB virus, measles virus, etc.) have a more positive role in stimulating the immune response. Viral epitopes and certain liver antigens have the same determinants, and they induce cross-reactions through "molecular simulation", leading to autoimmune liver disease. For example, some non-specific autoantibodies can be detected in the serum of some patients infected with hepatitis C virus. It is speculated that it is likely that the infection of hepatitis C virus changed the protein components on the liver cell membrane.

Clinical manifestations of autoimmune liver disease

In addition to the common clinical manifestations of autoimmune liver disease such as fatigue, decreased appetite, abdominal distension, discomfort in the liver area, and yellow staining of the skin and sclera, different types of diseases have their own characteristics.

The autoimmune hepatitis (AIH) lesions are mainly liver cells, which are more common in middle-aged women around 50 years old. They are characterized by elevated serum aminotransferase levels and serum immunoglobulin IgG (or -globulin). The level was significantly increased (> 20g / L), and autoantibodies such as serum antinuclear antibodies, antismooth muscle antibodies, antihepatic and renal microsomal type I antibodies, or antihepatocyte cytoplasmic type I antibodies were positive.

The primary lesions of primary biliary cholangitis (PBC) are interlobular bile ducts and small bile ducts, which are more common in women over 50 years of age. Clinically, itching may occur in the skin, and yellow tumors may appear in the upper crotch of the upper eye. Liver function tests are characterized by intrahepatic cholestasis, which is manifested as an increase in total bilirubin, which is higher than indirect bilirubin. Alkaline phosphatase and glutamyl transferase were significantly increased. Serum immunoglobulin IgM was significantly increased, and serum antinuclear antibodies and anti-smooth muscle antibodies were positive.

The lesions of primary sclerosing cholangitis (PSC) are mainly the intrahepatic bile ducts. A few can spread to the extrahepatic bile ducts. Most of the middle-aged men are around 40 years old. Most patients also have ulcerative colitis. Compared with primary biliary cholangitis, the incidence of primary sclerosing cholangitis is lower, but the clinical manifestations and laboratory tests are similar. Endoscopic retrograde cholangiography or percutaneous transhepatic cholangioangiography have Help the difference.

Overlap syndrome has the characteristics of the three diseases mentioned above.

Autoimmune liver disease test

1. Detection of various indicators of viral hepatitis: including hepatitis B virus-specific serum markers (commonly known as "two and a half"), HBVDNA, hepatitis C virus antibodies, and HCV RNA.

2. Immune indicators and autoantibody tests: Immune indicators include serum immunoglobulin IgM and IgG tests; autoantibody tests include serum antinuclear antibodies, antismooth muscle antibodies, antimitochondrial antibodies, anti-liver and kidney microsomal type I antibodies, and anti-hepatocyte Cytoplasmic type I antibodies.

3. Liver function test: evaluate liver function status.

4. Liver puncture pathological examination.

Diagnosis of autoimmune liver disease

The diagnosis of this group of diseases is complicated, and viral hepatitis, drug-induced hepatitis, alcoholic hepatitis, and metabolic liver disease must be excluded before diagnosis. Serum immunoglobulin levels and autoantibody tests are helpful for diagnosis, if necessary, pathological diagnosis by liver biopsy.

Treatment of autoimmune liver disease

1. Liver protection treatment can be treated with silymarin and polyene phosphatidylcholine.

2. Anti-inflammatory treatment can be treated with glycyrrhizic acid drugs, but glucocorticoids should be used with caution.

3. Cholesterol treatment can be treated with ursodeoxycholic acid and adenosylmethionine.

4. Liver failure patients, conditionally feasible allogeneic liver transplantation.