Abstract
Recalcitrant autoimmune hepatitis occurs in 7 % of patients treated with conventional corticosteroid regimens. High dose prednisone alone or a lower dose combined with azathioprine is the first line treatment. Doses are reduced after each month of improvement until clinical stability is achieved. Laboratory tests improve in 75 %, but histological resolution eventuates in only 20 %. Second line therapy with calcineurin inhibitors can be instituted for non-response or treatment intolerance, and mycophenolate mofetil is another option. Composite experiences indicate that 93-98 % of patients treated with cyclosporine or tacrolimus improve, whereas mycophenolate mofetil is effective in only 10 % with recalcitrant disease. Rituximab, rapamycin, non-mitogenic monoclonal antibodies to CD3, abatacept, and mesenchymal stem cell transplantation are plausible but untested rescue treatments. Problematic patients can be identified early by clinical phenotype, mathematical models, antibodies to soluble liver antigen, and rapidity of response to conventional corticosteroid treatment. Salvage therapies must not delay or supersede liver transplantation.
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