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Is Autoimmune Hepatitis Inherited?

Autoimmune hepatitis is associated with an uncontrolled activation of the immune system (the body’s defenses), which stops recognizing organs and tissues as their own and causes damage to one or several organs. The cause of autoimmune hepatitis is not known, however, it is known that there is a certain genetic predisposition to develop this disease, as with other autoimmune diseases.

Autoimmune hepatitis affects mostly young women, but can be seen at any age. The symptoms are variable, since the disease has a fluctuating course. It is not uncommon for the disease to be diagnosed by laboratory alterations in people who have no symptoms. There may be non-specific symptoms such as fatigue, nausea, abdominal pain and joint pains. Some patients may present as fulminating acute hepatitis, with significant jaundice, decreased prothrombin and encephalopathy. When the disease is more advanced, patients may present the symptoms of cirrhosis: jaundice, choluria, ascites, encephalopathy and variceal hemorrhage.

Some patients present extrahepatic manifestations of the disease, particularly autoimmune diseases of other organs such as thyroiditis, autoimmune hemolytic anemia, immune thrombocytopenic purpura, diabetes mellitus and polyglandular autoimmune syndrome. Autoimmune hepatitis is classified as:

Type 1 Autoimmune Hepatitis: It is the most frequent form and can be seen at any age. It usually presents with anti-nuclear (ANA) or anti-smooth muscle (ASMA) antibodies.

Type 2 Autoimmune Hepatitis: It occurs in children and adolescents. Its most characteristic marker is the presence of anti-microsomal liver-kidney antibodies (LKM-1).

There is no clinical manifestation or isolated laboratory examination that allows this disease to be diagnosed with certainty, and a combination of clinical, laboratory and histological criteria is used.

Laboratory findings include variable elevations of transaminases (SGOT / AST and SGPT / ALT) with normal or minimally elevated values of alkaline phosphatases and gamma glutamyl transpeptidase (GGT). There may be elevation of bilirubin, decrease in albumin and prolongation of prothrombin time. Characteristically, immunoglobulin G (IgG) levels are elevated. As part of the evaluation, serologic markers for viral hepatitis are negative.

The majority of patients present some autoantibody. The most frequent are:

ANA: Anti-nuclear antibodies (anti-nuclear antibodies).

ASMA: Anti-smooth muscle antibodies.

ALKM-1: Anti-microsomal antibodies of liver kidney (anti-liver kidney microsomal antibodies).

Other antibodies: Anti-asialoglycoprotein (ASGP), anti-actin (AAA), anti-soluble liver antigen (SLA), anti-neutrophil cytoplasm (ANCA).

The histology obtained in the liver biopsy is one of the main elements of the diagnosis, the characteristic findings being interface hepatitis and the presence of inflammation with plasma cells.

The diagnosis requires the exclusion of other causes of liver disease such as viral hepatitis, excessive alcohol consumption or exposure to hepatotoxic drugs. Diagnostic criteria have been developed to obtain a score and classify probable or definitive autoimmune hepatitis. The response to treatment is an important criterion of diagnosis.

Not all patients with autoimmune hepatitis require treatment. Certain criteria are accepted to begin treatment:

Transaminase about 10 times the maximum normal value.
Transaminase over 5 times the maximum normal value in the presence of gamma globulins (or IgG) over 2 times normal maximum value.
Bridge necrosis in liver biopsy.
Within the relative indications is the presence of symptoms (fatigue, arthralgia, jaundice) and interface hepatitis in the biopsy.
The majority of children should receive treatment at the time of diagnosis.

The treatment of autoimmune hepatitis is done with drugs that decrease the activation of immune cells. The most commonly used medications are corticosteroids (prednisone) and azathioprine.

The used medications can have adverse effects. Corticosteroids can produce changes in the shape of the face, increased appetite and the appearance of acne, as well as increasing the possibility of osteoporosis, diabetes and hypertension. Azathioprine is generally well tolerated, but is associated with neutropenia depending on the dose, in addition to the risk of pancreatitis and hepatotoxicity. The treatment should be closely monitored by a hepatologist.

Conclusion

The development of an autoimmune disease can be influenced by the genes that a person inherits and also the way in which the immune system of the person responds to certain triggers or environmental influences. It is known that some autoimmune diseases appear or worsen by certain triggers such as a viral infection; such is the case of autoimmune hepatitis.

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Is Autoimmune Hepatitis Inherited?