Aim: To assess the efficacy and safety of prolonged medical therapy for up to four decades in a cohort of patients with AIH.
Methods: Forty-two patients were followed long term in the Yale Liver Clinics who met the criteria of 'definite autoimmune hepatitis' as defined by the International Autoimmune Hepatitis Group. Records were reviewed for the dosage of immunosuppression, rate of relapse, steroid side effects, current status of liver function tests and evidence for cirrhosis and its complications.
Results: Mean follow-up was 16 years and ranged from 7 to 43 years. The median follow-up was 13.5 years. Steroid withdrawal resulted in a mean of 1.78 relapses/patient (range, 0-8). All but six patients responded well to prednisone and azathioprine and alanine aminotransferases were completely normal in 29 (81%) at last exam. Five patients have discontinued medication. Steroid side effects have been minimal (weight gain in eight, osteoporosis in three) except for one patient who recovered successfully from cryptococcal meningitis and another with aseptic necrosis of the hip. Progression to cirrhosis occurred in 54% with evidence of esophageal varices in 37% but none developed hepatocellular carcinoma. Only one patient has received a liver transplant, while five others are currently listed because of symptoms of ascites, encephalopathy or bleeding from esophageal varices.
Conclusions: AIH can be managed effectively over three to four decades with low-dose immunosuppression resulting in essentially normal lifestyles and minimal side effects. Liver transplantation with an increased risk of rejection and graft failure in this group can be avoided for long periods in most of these patients.