Autoimmune hepatitis is a form of chronic liver disease that is very common in our area, usually affecting young women. Often, however, its diagnosis is delayed or missed as it does not have specific symptoms or laboratory tests. Autoimmune hepatitis is a result of alterations in the body’s immune system that lead to the production of specific factors that attack liver cells. The exact cause of the disease has not been yet determined but its course and treatment are well described.
Autoimmune hepatitis classically affects young woman in their second and third decade of life. Having said this, however, this condition may present at any age. People with autoimmune hepatitis may seek care because they have been noted to have high liver chemistries or even mildly elevated liver tests. About fifty percent of people will present with liver chemistries in the high hundreds or even in the thousands. This scenario is no different than the presentation of any form of acute hepatitis including hepatitis A and B and any drug-induced hepatitis. A person typically complains of fatigue, loss of appetite and weight loss. The eyes and skin may become jaundiced or yellow. These episodes of abnormal liver tests can spontaneously resolve and recur at a later time. Despite that fact that there are no specific markers for autoimmune hepatitis, there are certain blood tests that are typical of this condition. The serum total protein is usually very high and the serum immunoglobulins (chemicals made by the body as part of the immune system response) are also elevated. Autoantibodies such as the anti-smooth muscle antibody are usually positive. These tests are generally sufficient to make the diagnosis of autoimmune hepatitis and once the diagnosis is made, therapy should be initiated.
A liver biopsy remains an important component in the evaluation of autoimmune hepatitis. It helps with diagnosis but more importantly it is critical in assessing prognosis as people who develop cirrhosis at an early age are more likely to require a liver transplantation in the future. About fifty percent of people who first show signs of autoimmune hepatitis will already have cirrhosis on a liver biopsy. This is important for both the patient and physician to know. Classically, a the liver biopsy in autoimmune hepatitis is filled with plasma cells, the cells in the blood stream which are involved in the body’s immune system.
The textbook describes the classic person with autoimmune hepatitis as a young woman with a history of acne, an abnormal menstrual cycle and hirsuitism. Most patients, however, do not follow the textbook and simply have the blood tests consistent with the disease. As this is an autoimmune disease, there are many other autoimmune conditions associated with it including arthritis, skin diseases, kidney disease, lung disease, thyroid disease and anemia, to name just a few.
The treatment of autoimmune hepatitis is aimed at modulating the immune system. Therefore, the initial treatment is a steroid such as prednisone that is very slowly tapered over a long time course. As steroids have significant side effects, especially when given over a long period of time, other immune modulators such as azothioprine or mycophenolate are given in an effort to reduce steroid dosages. If therapy is unsuccessful and the patient develops complications of cirrhosis, liver transplantation is an option in these patients although the condition may recur in the new liver. The key to treating autoimmune hepatitis is early diagnosis so it is important to remember this common condition and to adequately assess for it.