The immune system’s job is to protect the body against harmful substances or diseases but, on occasion, it malfunctions and starts to attack some of its own tissues and organs. Examples of the more common autoimmune diseases include insulin-dependent diabetes (pancreas is under attack), Grave’s disease (thyroid is affected), rheumatoid arthritis (joints are attacked), and ulcerative colitis (colon is affected). In the case of autoimmune hepatitis (AIH), the organ under siege is the liver.
Like most autoimmune disorders, AIH occurs predominantly in women. Why AIH occurs is unclear, but it is believed that it could be triggered in persons who are genetically predisposed to developing an immune disorder, or by viruses (e.g., hepatitis A, B, or C, and measles), bacteria (e.g., salmonella and E. coli), medications (e.g., Aldomet® (methyldopa), Macrobid® (nitrofurantoin), and Minocin® minocycline)), and possibly even herbs (e.g., Dai-saiko-to and black cohosh). The condition is chronic and progressive, meaning that it can lead to cirrhosis and death if left untreated.
Signs and symptoms of AIH can range from minor to severe and may come on suddenly or develop gradually over time. One in five cases are discovered by accident, as a patient may have no symptoms and unexplained elevated liver enzyme levels on a routine blood test. On the other end of the spectrum, AIH may present as an acute attack with extremely high liver enzyme levels, yellowing of skin and eyes (jaundice), severe itching, pain in the right upper quadrant of the abdomen, and fatigue. The acute illness may appear to resolve spontaneously; however, patients invariably develop signs and symptoms of chronic liver disease, which include fatigue, loss of appetite, muscle and joint pain, diarrhea, and skin rashes.
Physicians typically diagnose AIH by using a series of specialized blood tests that distinguish AIH from other causes of hepatitis and other disorders with similar symptoms. A liver biopsy is usually performed to confirm the diagnosis and to determine the degree of damage to the liver. It is a procedure where the physician or surgeon removes a small amount of the liver tissue by inserting a thin needle quickly between the ribs and then examining the sample under a microscope.
Treatment works best when AIH is diagnosed early. The goal in treating AIH is to slow or stop the body’s immune system from attacking the liver. The medications used are immunosuppressants, such as prednisone and Imuran® (azathioprine). Physicians usually prescribe a high initial dose of prednisone, and then taper it down progressively as symptoms and liver enzymes improve. Most people will need to take medication for the rest of their lives. Since prednisone can cause a wide range of side effects, Imuran® is often used in conjunction to allow for a lower dose of the prednisone.
Some people may go into remission, during which physicians can effectively discontinue treatment; others will relapse after stopping treatment, and will then need to restart the medication and continue on long-term maintenance therapy. A few patients may eventually be tapered off the prednisone completely and stay solely on Imuran®. For those who do not respond to, or relapse from, the combination regimen, then stronger immunosuppressive agents such as mycophenolate mofetil, cyclosporine, or tacrolimus may be considered. When medications do not halt the progress of the disease, or complications from cirrhosis have developed, the remaining option is a liver transplant. Fortunately, the success rate of transplantation in people with AIH is excellent.
With proper treatment, autoimmune hepatitis can often be controlled, and in those that have a sustained response to treatment, the progression of the disease lessens and some of the damage may be reversed. Therefore, it is possible to have a normal life expectancy, even with the life-long condition of autoimmune hepatitis.