Autoimmune hepatitis (AIH) was the first chronic
liver disease which responded favorably to
drug therapy. It has a dismal prognosis when not treated. Since the original description in 1950 by Waldenström the treatment option initially reported is still practiced today and is the core of the basic therapeutic strategy of inducing remission with
steroids and
azathioprine. It is important to establish the diagnosis before
cirrhosis develops. Later, the avoidance of
immunosuppressant side effects, nonresponders to standard induction
therapy, and adherence to
therapy are among the greatest challenges in treating AIH. Alternative immunosuppressive drugs have been tested in small series and have included commonly used transplant
immunosuppressants, albeit with mixed results and many unwanted
drug side effects. A recent large multicenter prospective treatment trial suggests that
budesonide may offer an alternative in noncirrhotic AIH patients capable of minimizing unwanted
steroid effects. The ultimate treatment approach upon
drug treatment failure is
liver transplantation. Only 4% of transplant candidates are transplanted for AIH. After
liver transplantation, there is a considerable risk for graft loss because of recurrent AIH, and lifelong vigilance and therapeutic attention is important.