The nature and prognostic implications of autoimmune hepatitis with an acute presentation

To determine the nature and prognostic implications of autoimmune hepatitis with an acute presentation, 12 patients with a disease duration of 3 months or less (mean duration, 2.3 +/- 0.2 months) were compared to 14 patients with a disease duration of 12 months or more (mean duration, 15.1 +/- 0.9 months). Liver tissue specimens were graded under code for lobular, portal and architectural changes. Patients with acute and chronic presentations were indistinguishable by age, sex, human leukocyte antigen phenotype, immunoserologic markers, and biochemical indices of liver inflammation. Moderate to severe lobular hepatitis was present more frequently in patients with acute presentations (75% versus 29%, p = 0.2), but differences were not statistically significant. Bridging fibrosis and cirrhosis were seen with equal frequency in both groups (79% versus 73%). Remission, relapse, treatment failure, progression to cirrhosis, and death from hepatic failure occurred with similar frequencies in patients with acute and chronic presentations. We conclude that autoimmune hepatitis with an acute presentation is indistinguishable by clinical and laboratory features from that with a chronic presentation and it is probably a pre-existent subclinical disease that is unmasked by disease progression or an abrupt exacerbation. Lobular hepatitis is an important histologic feature regardless of disease duration. The response to corticosteroid therapy is unaffected by the perceived duration of disease prior to treatment.