In patients with autoimmune hepatitis (AIH), immune cells destroy specialized liver cells known as hepatocytes.
The disease affects young women most commonly. Some with AIH may be genetically predisposed to getting the disease. Their immune systems may be overly sensitive to certain infectious agents or toxic environmental chemicals. These, in turn, may act as triggers for autoimmune disease.
Diagnosis
Laboratory blood work that measures a variety of antibodies and enzymes is usually sufficient to make a positive diagnosis of AIH. First, however, other liver diseases such as viral hepatitis, which is treated differently, need to be ruled out as causes. Imaging of the liver is also important to rule out possible mechanical liver problems. This is easily accomplished with a noninvasive magnetic resonance imaging (MRI) technique known as MRCP (Magnetic Resonance Cholangiopancreatography). In some cases, an endoscopic procedure known as ERCP (Endoscopic Retrograde Cholangiopancreatography), or a liver biopsy, may be required.
In AIH, high levels of antibodies specific for cell proteins, called anti-nuclear antibodies, are observed, as well as high levels of Immunoglobulin G (IgG). This is the same protein that, when elevated, means an allergic response such as a nasal allergy or an infection has occurred.
When a patient with AIH undergoes liver biopsy, inflammation along the border of liver lobules - the basic functional units of the liver responsible for organ nourishment and removal of cell waste -- is visible and helps confirm the diagnosis.
Treatment
AIH patients are treated with a combination of steroids and azathioprine. Both of these slow immune system function and stop the progress of liver cell damage. However, roughly half the patients who respond to steroids might experience a flare-up in later years. Clinical trials - in which promising new drugs and treatments are tested for use in AIH - are often conducted at Mount Sinai, giving our patients access to experimental treatments. For example, not all patients respond to azathioprine or tolerate it well, so Mount Sinai was recently involved in a multi-center clinical trial to test a new drug as a possible substitute.
Physicians and researchers at Mount Sinai are also undertaking research projects to obtain a better understanding of the autoimmune response in chronic liver diseases, how the conditions progress, and what treatments might stop or control the disease process.
