Long-term management and prognosis of autoimmune hepatitis (AIH): A singlecenter experience

Background: Controlled trials have firmly established the need for immunosu ppressive therapy in autoimmune hepatitis.. However, reports about long-ter m management and prognosis of the disease are scarce. Patients and methods: We reviewed the charts of 103 consecutive patients wi th a well-documented long-term course of autoimmune hepatitis who had been carefully managed over a mean observation period of 95 months (12-405 month s). Results: Under immunosuppressive therapy 94 patients (91.2%) reached comple te remission after a mean treatment duration of 3 +/-3 months. 28 of the 10 3 patients (27.2%) were eligible for a trial of treatment withdrawal after a mean treatment duration of 32.2 months (range: 12-81 months). 21 of these patients (75%) had a relapse following treatment withdrawal. 13.6% of pati ents had intolerance of or severe side effects to azathioprine. There was n o increase in tumor risk during a cumulative observation period of 423 pati ent-years of azathioprine therapy. Corticosteroid side effects occurred mos tly during induction therapy, but were usually minor and resolved upon dose reduction. During a cumulative observation period of 842 patient-years no liver related deaths occurred and no patient had to be referred to liver tr ansplantation, even though 30 patients (29.1%) had histological evidence of cirrhosis at presentation. The overall 5-and 10-year survival of patients with autoimmune hepatitis was identical to an age- and sex-matched control population. Conclusion: Our study shows that the majority of patients with AIH do achie ve a complete remission within 3 months, but require long-term or permanent immunosuppressive therapy that is usually well tolerated. Long-term surviv al in well-managed patients is excellent.