Recent-onset autoimmune hepatitis. Biopsy findings and clinical correlations.

Whereas the histologic findings in clinically "chronic" autoimmune hepatitis have been well established, with piecemeal necrosis as a hallmark lesion, the histologic findings of clinically "acute" or recent-onset autoimmune hepatitis remain undefined. The goal of this study was to define more fully the liver histomorphology in patients with recent-onset autoimmune hepatitis. Twenty-six patients were identified at our institution who had well-characterized autoimmune hepatitis and had undergone a liver biopsy within 6 months of clinical presentation. A detailed histologic evaluation revealed evidence of chronic liver disease in 25 (of 26) patients despite the lack of correlating clinical chronicity. The histologic evidence of chronicity included, in addition to a portal lymphoplasmacytic infiltrate, bridging (septal) fibrosis (11 patients) and overt cirrhosis (four patients). Eighteen of these 25 cases with evidence of chronicity also showed zone 2 and 3 lobular hepatitis, including disarray and hepatocyte necrosis. A single case showed lobular hepatitis with confluent hepatocyte necrosis (submassive hepatocellular necrosis), but no evidence of chronic liver disease. Although autoimmune hepatitis remains in the differential diagnosis of lobular hepatitis, these data show that most patients with autoimmune hepatitis who undergo biopsy early in its clinical course will have histologic evidence of chronic liver disease. Most of these patients probably have a lobular "flare" in disease activity, which likely precipitated the clinical presentation. The findings herein reinforce the concept that autoimmune hepatitis is by definition a chronic disease and supports the proposal that the modifier "chronic" be eliminated from autoimmune hepatitis.

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.

Liver transplantation for jejunoileal bypass-associated cirrhosis: allograft histology in the setting of an intact bypassed limb.

Jejunoileal bypass (JIB) is a well known cause of steatohepatitis, which may, on occasion, progress to cirrhosis and require liver transplantation. We report 3 patients who underwent orthotopic liver transplantation (OLT) for steatohepatitic cirrhosis secondary to JIB in which the JIB was left intact. All 3 patients have demonstrated recurrent steatosis in the graft after liver transplantation. In two of the cases, the changes are moderately severe, whereas in one case the changes are mild. All 3 patients have essentially normal liver function tests and are clinically asymptomatic; 1 of the patients has undergone removal of the JIB 2.5 years after transplantation. Control hepatic allografts in patients with primary biliary cirrhosis and primary sclerosing cholangitis show negligible fatty change, and in patients who receive transplants for alcoholic steatohepatitis, they rarely (2 of 20 patients) contain greater than 10% fat. We conclude that transplantation alone is not associated with subsequent steatosis. Presence of JIB is, therefore, a continuing risk factor for steatosis in patients who have undergone OLT. Reversal of JIB after OLT may be considered if fatty changes are severe or associated with significant fibrosis.