Material and methods: With the aim of analyzing the influence of presence of cirrhosis at baseline on the outcome, we revised the evolution of a cohort of patients with type 1 autoimmune hepatitis, prospectively followed at a single hospital. 139 patients (113 females, 26 males), median age 45.7 years, interquartile range 13-59 years, were followed-up for a median period of 58 months (interquartile range 27-106).
Results: At baseline, 55 patients had cirrhosis and they were significantly older, had lower prothrombin activity and serum albumin than patients without cirrhosis. In contrast, patients without cirrhosis had significantly higher bilirubin, AST and ALT levels at diagnosis time. There was no significant difference in the follow-up time between patients with and without cirrhosis at baseline and either in the percentage of patients receiving immunosupresor treatment (80 vs. 91%, respectively) or in the response to therapy (complete response in 82 vs. 95%, respectively). However, patients with cirrhosis had a significantly lower probability of remaining free of cirrhosis complications (49.1% at 102 months, 95%CI, 35.5-67.9% vs. 86.7%, 95%CI, 77.1%-97.5%, respectively) (p = 0.0000) and a significantly lower overall survival at 120 months (67.1%, 95%CI, 51.3-87.6 vs. 94.4%, 95%CI, 86.9-100%, respectively) (p = 0.003) than those without cirrhosis at presentation.
Conclusion: Patients with type 1 autoimmune hepatitis and cirrhosis at presentation have a lower survival than those without cirrhosis despite a similar response to treatment.