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Is Autoimmune Hepatitis a Rare Disease?

Autoimmune hepatitis is a chronic, infrequent and of unknown cause liver disease. The cause is an alteration of the patient´s immune response, which destroys the liver cells and inflames the liver. It usually has a chronic course with potentially serious outbreaks of activity. If the situation continues, a process of scarring or fibrosis appears in the liver that leads to cirrhosis and liver failure. The prognosis of the disease improves a lot when patients are treated with drugs that decrease the immune response and inflammation (immunosuppressants).

It can affect all ages and both sexes, but it is more common in women between 10 and 50 years old. In more than 40% of cases, it presents as an episode of acute hepatitis, including fulminating hepatitis, and this usually happens more in children or young people. The possible symptoms are malaise, tiredness, lack of appetite, jaundice (yellowish coloration of skin and mucous) and alterations in menstruation. At other times, it does not produce symptoms or only a feeling of tiredness and the disease is discovered by the presence of elevated transaminases (liver enzymes) in an analysis done for another reason. It is sometimes discovered when liver cirrhosis has developed due to its complications, such as ascites (accumulation of fluid in the abdomen) or digestive hemorrhage due to esophageal varices. Also the diagnosis can be reached when studying patients with other autoimmune diseases.

Diagnosis

It requires excluding other causes of liver disease, such as viral hepatitis (Hepatitis A, B or C), damage by drugs, toxics or alcohol, metabolic diseases such as nonalcoholic steatohepatitis, and metal deposition diseases (hereditary) such as Wilson’s disease or hemochromatosis. The diagnosis is established by the laboratories data, the biopsy and the patient’s symptoms. In the analytical studies, elevated transaminases stand out, sometimes as if they were acute hepatitis. The presence of elevated immunoglobulins (hypergammaglobulinemia) is especially constant, especially with elevation of the so-called IgG. High titers of autoantibodies (antibodies produced by the organism against itself) are detected, such as antinuclear antibodies (ANA), anti-smooth muscle antibody (ASMA) or antimicrosomal antibodies of the liver and kidney (anti LKM-1).

These autoantibodies are considered diagnostic markers of the disease, but are not specific to it. Furthermore, there is no correlation between the titer or elevation of autoantibodies and the severity of autoimmune hepatitis. In the study of the disease, it is advisable to perform a liver biopsy, which usually highlights the high inflammatory activity and abundant plasma cells within the liver tissue. The diagnostic suspicion of autoimmune hepatitis is supported by the fact that patients have another associated autoimmune disease (autoimmune thyroiditis, for example) and also the favorable response to immunosuppressive treatment.

Treatment

The goal of treatment is preventing the progression of the disease. The specialists agree that the initial treatment of this disease will be with corticosteroids (prednisone, methylprednisolone), at varying doses, to achieve remission of the disease. Corticosteroids have side effects, some only cosmetics (face of a full moon, appearance of body hair, thick abdomen) and other more relevant ones such as decreased bone mass (osteoporosis), diabetes and high blood pressure, among others. The majority yield by stopping the drugs. To avoid these side effects, treatment with corticosteroids at lower doses than usual can be initiated, associating another immunosuppressant drug, such as azathioprine. Once remission or inactivation of the disease has been achieved, corticosteroids will be discontinued, continuing azathioprine as the best maintenance treatment to prevent reactivation. Azathioprine can also have side effects such as bone marrow depression, so it is essential to periodically perform blood tests (white and red blood cells, platelets, transaminases, etc), while the treatment lasts.

Conclusion

Autoimmune hepatitis is an infrequent disease of insidious course characterized by chronic inflammation of the hepatic parenchyma of unknown etiology. In its etiopathogenesis, environmental triggers and lack of tolerance seem to be influenced in genetically predisposed patients that lead to the attack of the liver parenchyma by lymphocytes T.

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Is Autoimmune Hepatitis a Rare Disease?