Background: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder of unknown aetiology, which when left untreated can lead to liver cirrhosis and hepatic failure. Current treatment strategies include long-term treatment with corticosteroids and/or azathioprine. Most patients respond well to immunosuppressive therapy and treatment usually results in an asymptomatic course of AIH in remission. Nevertheless, both drugs are associated with serious side effects that can sometimes be severe and may necessitate drug withdrawal. Whether or not treatment in patients who are in longstanding remission can be discontinued is unknown. Key Messages: Available data rely on retrospective data sets and are not conclusive. Some studies indicate that a sustained remission after treatment withdrawal is feasible, whereas other studies have found relapse rates in up to 90% of patients, even in patients with established histological remission. Patients who relapse after drug withdrawal have a high probability for a re-relapse to occur. Life-long maintenance therapy should be strongly considered in these patients, since patients who have multiple relapses are more likely to progress to cirrhosis, liver transplantation and death from liver failure. Conclusion: For a majority of patients, AIH is a lifelong disease requiring permanent treatment. Patients in longstanding clinical remission on monotherapy, with complete normalisation of aminotransferases and IgG could be offered one attempt of drug withdrawal. The risk of disease progression after a single relapse appears low, while a patient's realization that infinite maintenance therapy is mandatory may improve drug adherence.

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