Autoimmune hepatitis is a condition in which the patient's own immune systems attacks the liver causing inflammation and liver cell death. The condition is chronic and progressive. Although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis.
"Autoimmune hepatitis usually occurs in women (70 %) between the ages of 15 and 40. Although the term "lupoid" hepatitis was originally used to describe this disease, patients with systemic lupus erythematosus do not have an increased incidence of autoimmune hepatitis and the two diseases are distinct entities. Patients usually present with evidence of moderate to severe hepatitis with elevated serum ALT and AST activities in the setting of normal to marginally elevated alkaline phosphatase and gamma-glutamyltranspeptidase activities. The patient will sometimes present with jaundice, fever and right upper quadrant pain and occasionally systemic symptoms such as arthralgias, myalgias, polyserositits and thrombocytopenia. Some patients will present with mild liver dysfunction and have only laboratory abnormalities as their initial presentation. Others will present with severe hepatic dysfunction." -Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of Medicine. 334:897-903.
Autoimmune hepatitis is a chronic inflammatory disease of the liver. Amino transferases are elevated in patients who have had viral metabolic and toxic events ruled out.
Autoimmune hepatitis was previously called "lupoid" hepatitis. It was originally described in the early 1950s.
Most patients do have an associated autoimmune disorder such as systemic lupus erythematosis. Thus, its name was previously lupoid hepatitis.
Because the disease has multiple different forms, and is not always associated with systemic lupus erythematosis, lupoid hepatitis is no longer used. The current name at present is autoimmune hepatitis (IAH).
Patients who have chronic elevations in liver function tests for greater than 6 months are thought to have chronic hepatitis. In these individuals, other diseases are evaluated. When Wilson's disease, hemochromatosis, alpha 1 antitrypsin deficiency, Hepatitis B and C and drug induced hepatitis have been ruled out, a possible autoimmune disorder is present.
Autoimmune disorders are divided according to the type of antibody that is present. In general, there are four major classifications. These are ANA (anti-nuclear antigen), SMA (smooth muscle antigen), and anti LKM (anti liver kidney miscrosomal) positive subtypes.
Evaluation for these types of autoimmune hepatitis is obtained through simple blood tests.
Based upon the serology and associated rheumatologic disorders, a patient may be treated with Prednisone, etc.
Indications for Autoimmune Chronic Hepatitis Treatment
Not all patients with autoimmune hepatitis require treatment. However, when they have serum amino transferase elevations greater than 10 times above normal, scarring of the liver, and/or other features associated with hepatic failure, treatment is indicated. Usually, patients are treated with Prednisone 40 mg po qd. Current therapy may include 6 mercaptopurine with or without steroids.
In general, patients do quite well with autoimmune hepatitis. Treatment is usually required several years in order to bring the liver function tests, hepatic inflammation and changes into more normal perimeters.
Most of the improvement in liver function tests occurs within the first 6 months of treatment. However, changes in the liver histology do require longer-term therapy. This usually occurs 6-12 months after liver function tests have improved. Thus, long-term treatment is required for good control of autoimmune hepatitis patients.