Autoimmune hepatitis with incidental histologic features of bile duct injury

Abstract

Bile duct changes are atypical of autoimmune hepatitis. Our aims were to assess the frequency and significance of these changes in classical disease. Liver biopsy specimens were reviewed under code from 84 patients who satisfied international scoring criteria for autoimmune hepatitis, and the findings were correlated with clinical features and outcome. Twenty patients (24%) had biliary changes, including 6 with destructive cholangitis, 4 with ductopenia, and 10 with nondestructive cholangitis. Patients with and without bile duct changes had similar laboratory findings. Diagnostic scores for autoimmune hepatitis were lower in patients with bile duct changes (16.6 ؎ 0.6 vs. 19.1 ؎ 0.2, P < .0001). The frequencies of scores sufficient for a definite (80% vs. 97%, P ‫؍‬ .03) or probable diagnosis (20% vs. 3%, P ‫؍‬ .03) were also less in this group. Patients with destructive cholangitis and/or ductopenia responded as well to therapy as patients with nondestructive cholangitis, and outcomes in each group were similar to those of patients without biliary changes. We concluded that biliary changes can occur in classic autoimmune hepatitis, and they are not associated with distinctive clinical features or treatment response.

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: A prospective magnetic resonance imaging and histological study

“…22 Histological evidence of destructive/nondestructive cholangitis was found in 12 (20%) patients. This finding is in keeping with the results of Czaja and Carpenter, 20 who found such bile duct lesions in 16 of 84 biopsies obtained from patients with AIH. No case of bile duct loss was observed.…”

“…Interlobular bile duct paucity was defined as a ductopenia of Ͼ 50%. 20 Magnetic Resonance Imaging. Magnetic resonance examinations were obtained with a 1.5-T system (1.5-T Magnetom Maestro Class Symphony, Siemens Medical Systems, Erlangen, Germany and Gyroscan Intera, Philips Medical Systems, Best, the Netherlands) using a phased array abdominal coil for signal reception.…”

Stromal Cell–Derived Factor-1 from Biliary Epithelial Cells Recruits CXCR4-Positive Cells: Implications for Inflammatory Liver Diseases

“…The accumulation of inflammatory cells around the bile duct is a characteristic feature of primary biliary cirrhosis (PBC), but the same can be seen in livers of viral hepatitis or AIH (Czaja and Carpenter, 2001;Kaji et al, 1994;Ludwig et al, 1984). The accumulation of inflammatory cells around the bile ducts in PBC has been explained as immunologic reactions against biliary epithelial cells (BEC).…”

Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

“…The King's liver unit reported the presence of histological and/or radiological biliary features compatible with PSC in 50% of children who otherwise fulfilled criteria for AIH [55]. Czaja and Carpenter [56] reported that 20 of 84 adult patients with AIH had biliary changes on liver biopsy in the absence of anti-mitochondrial autoantibodies. In other respects, they behaved like AIH and responded to immunosuppressive therapy [56].…”

“…Czaja and Carpenter [56] reported that 20 of 84 adult patients with AIH had biliary changes on liver biopsy in the absence of anti-mitochondrial autoantibodies. In other respects, they behaved like AIH and responded to immunosuppressive therapy [56]. Current evidence suggests that PSC may be preceded in some patients by AIH, particularly in children and adolescents.…”