What is Autoimmune Hepatitis?

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What is Autoimmune Hepatitis? Autoimmune hepatitis, or autoimmune chronic hepatitis, is a progressive inflammation of the liver that has been identified by a number of different names, including autoimmune chronic active hepatitis (CAH), idiopathic chronic active hepatitis, and lupoid hepatitis. The reason for this inflammation is not certain, but it is associated with an abnormality of the body's immune system and is often related to the production of antibodies that can be detected by blood tests.Autoimmune hepatitis was first described in 1950 as a disease of young women and is associated with increased gamma globulin in the blood and chronic hepatitis on liver biopsy. The presence of antinuclear antibodies (ANA) and the resemblance of some symptoms to "systemic lupus erythematosus" (SLE) led to the label "lupoid hepatitis." It later became evident that this disease was not related to SLE. The disease is now called autoimmune hepatitis.What are the Symptoms?The typical patient with autoimmune hepatitis is female (70%). The disease may start at any age, but is most common in adolescence or early adulthood. Blood tests identify ANA or smooth muscle antibodies (SMA) in the majority of patients (60%). More than 80% of affected individuals have increased gamma globulin in the blood. Some patients have other autoimmune disorders such as thyroiditis, ulcerative colitis, diabetes mellitus, vitiligo (patchy loss of skin pigmentation), or Sjogren's syndrome (a syndrome that causes dry eyes and dry mouth). Other liver diseases such as viral hepatitis, 's disease, hemochromatosis, and alpha-1-antitrypsin deficiency should be excluded by appropriate blood tests, and the possibility of drug-induced hepatitis is ruled out by careful questioning.The most common symptoms of autoimmune hepatitis are fatigue, abdominal discomfort, aching joints, itching, jaundice, enlarged liver, and spider angiomas (blood vessels) on the skin. Patients may also have complications of more advanced chronic hepatitis with cirrhosis, such as ascites (abdominal fluid) or mental confusion called encephalopathy. A liver biopsy is important to confirm the diagnosis and provide a prognosis. Liver biopsy may show mild autoimmune hepatitis, more advanced chronic hepatitis with scarring (fibrosis), or fully developed cirrhosis.How is Autoimmune Hepatitis Treated?The ten-year survival rate in untreated patients is approximately t10%. The treatment of autoimmune hepatitis is immunosuppression with prednisone alone or prednisone and azathioprine (Imuran). This medical therapy has been shown to decrease symptoms, improve liver tests, and prolong survival in the majority of patients. Therapy is usually begun with 30 to 40 mg per day of prednisone. This dosage is reduced after a response is achieved.The standard dosage used in the majority of patients is 10-15 mg of prednisone per day, either alone or with 50 mg of azathioprine per day. Higher doses of prednisone given long-term are associated with an increase in serious side effects, including: hypertension, diabetes, peptic ulcer, bone thinning, and cataracts. Lower doses of prednisone may be used when combined with azathioprine.The goal of treatment of autoimmune hepatitis is to cure or control the disease. In two-thirds to three-quarters of the patients, liver tests fall to within the normal range. Long-term follow-up studies show that autoimmune hepatitis appears more often to be a controllable rather than a curable disease, because the majority of patients relapse within six months after therapy is ended. Therefore, most patients need long-term maintenance therapy.Not all patients with autoimmune hepatitis respond to prednisone treatment. Approximately 15-20% of patients with severe disease continue to deteriorate despite initiation of appropriate therapy. This is most common in patients with advanced cirrhosis on initial liver biopsy. Such patients are unlikely to respond to further medical therapy, and liver transplantation should be considered.The information contained in this sheet is provided for information only. This information does not constitute medical advice and it should not be relied upon as such. The American Liver Foundation (ALF) does not engage in the practice of medicine. ALF, under no circumstances, recommends particular treatments for specific individuals, and in all cases recommends that you consult your physician before pursuing any course of treatment.*The American Liver Foundation gratefully acknowledges the contribution of D. Lindor, MD, who reviewed and updated this information.

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Autoimmune HepatitisBy J. Worman, M. D.

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 distint 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. Autoimmune hepatitis should be suspected in any young patient with hepatitis, especially those without risk factors for alcoholic, drug, metabolic or viral etiologies. Serum protein electrophoresis and testing for autoantibodies are of central importance in the diagnosis of autoimmune hepatitis. Patients with one subtype of autoimmune hepatitis have serum gamma-globulin concentrations more than twice normal and sometimes antinuclear antibodies and/or anti-smooth muscle (anti-actin) antibodies. Patients with another subtype may have normal or only slightly elevated serum gamma-globulin concentrations but will have antibodies against a particular cytochrome p450 isoenzyme that are called anti-LKM (liver kidney microsome). Patients in whom a diagnosis of autoimmune hepatitis is suspected should have a liver biopsy. If the biopsy is consistent, treatment with steroids (prednisone or pednisolone) and azathioprine (Imuran) is begun immediately. These are tapered over the next 6 to 24 months depending upon the patient's course. If immediate liver biopsy is contraindicated because of a prolonged prothrombin time or thrombocytopenia, steroids and azathioprine should be started prior to biopsy if the diagnosis of autoimmune hepatitis is likely based on clinical criteria (e.g. a young woman with severe hepatitis, elevated serum gamma-globulin concentration, negative risk factors and serologies for viral hepatitis). The patient will often rapidly improve and biopsy should be performed to confirm the diagnosis as soon as the prothrombin time decreases and platelet count increases to within safe ranges. About two thirds to three quarters of patients with autoimmune hepatitis respond to treatment based on the return of serum ALT and AST activities to normal and an improved biopsy after several months. Some patients relapse as steroids and azathioprine doses are tapered or stopped and need chronic maintenance medications. Over the long term, many patients develop cirrhosis despite having a response to treatment, and patients who do not respond to treatment will almost always progress to cirrhosis. If end-stage liver disease develops, orthotopic liver transplantation is an effective procedure. For more information, you may want to see the following review articles: Czaja, A.J. and Freese, D. K. 2002. Diagnosis and treatment of autoimmune hepatitis. Hepatology. 36:479-497. Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of Medicine. 334:897-903.