Prognosis of histological cirrhosis in type 1 autoimmune hepatitis

Background & Aims: Cirrhosis connotes irreversible damage to the liver and a shortened life expectancy. The aim of this study was to evaluate the impact of cirrhosis on treatment response and survival in type 1 autoimmune hepatitis. Methods: One hundred twenty-eight patients were evaluated for histological cirrhosis. Response to treatment, predictors for cirrhosis, and outcomes were determined. Results: Thirty-seven patients (29%) had histological cirrhosis at entry, whereas 36 of the 91 patients without cirrhosis (40%) developed it during 39 +/- 32 months. Development of cirrhosis was predicted by lower serum albumin levels (P = 0.04) and higher prothrombin times (P < 0.001) at presentation. The frequencies of remission, relapse after drug withdrawal, and treatment failure were comparable in patients with and without cirrhosis at entry. The overall 10-year survival (93%) was similar to that of an age- and sex-matched cohort from the population at large (94%). Ten-year survival was not different between those with and those without cirrhosis at entry (89% and 90%, respectively), even with transplantation as an end point. Conclusions: Patients with histological cirrhosis at presentation respond to corticosteroid therapy as well as patients without cirrhosis. Low serum albumin levels and hypoprothrombinemia at presentation predict the development of cirrhosis. Histological cirrhosis does not diminish survival expectations.