Maggio 2002 - Volume XXI - numero 5
Focus
Dipartimento di Medicina della Procreazione e dell’Età Evolutiva, Scuola di Specialità in Pediatria, Università di Pisa
Key words: Autoimmune hepatitis, Interface hepatitis, Immunosuppressive therapy, Cyclosporine, Liver transplantation
Autoimmune hepatitis (AIH) is an uncommon disease occurring mainly in women and characterised by the morphological changes of interface hepatitis on liver biopsy, hypergammaglobulinemia, elevated serum aminotransferases, and circulating autoantibodies. AIH is thought to have a basis in aberrant autoreactivity to hepatocyte antigens in genetically predisposed individuals. AIH is notably heterogeneous with respect to its clinical expression and laboratory features and patients may present without obvious clinical evidence of liver disease or with an acute hepatitis. Corticosteroids alone or in conjunction with azathioprine are the treatment of choice and result in remission induction in over 90% of patients. Sustained response to therapy may result in substantial regression of fibrosis even in advanced cases. Rapid withdrawal of immunosuppression is associated with high risk of disease relapse in many patients. Alternative strategies in patients who have failed to achieve remission on “standard therapy” include the use of cyclosporine. Liver transplantation is the treatment of choice in managing patients with decompensated disease unresponsive to “rescue” medical treatment.
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