Cited by 189Liver Diseases and ImmunityIgG4-Related and Inflammatory DiseasesPediatric Hepatobiliary Diseases and Treatments

Autoimmune hepatitis with incidental histologic features of bile duct injury

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Czaja, Albert J., Carpenter, Herschel A.·2001·Hepatology

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. They may be coincidental findings associated with classic disease or weak expressions of a variant syndrome. In the absence of a cholestatic clinical syndrome, they do not compel a different management strategy.

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Crossref · 10.1053/jhep.2001.27562

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  • Acute AIH may be accompanied by considerable bile duct damage.

    “as well as that acute AIH may be associated with considerable bile duct damage [170].”

    From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [170]

    The Discussion reports that bile duct injury, including destructive cholangitis and ductopenia, can occur in classic autoimmune hepatitis. The Frequency of Cholangitis and Ductopenia section reports these findings in specimens obtained at presentation, but the paper does not establish that those patients had acute-onset AIH; presentation is not the same as acute disease. (LLM, conf=0.85)

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  • AMA can be present in acute hepatitis, with titres usually falling over time.

    “AMA may be present in patients with acute hepatitis, with titres usually decreasing over time;”

    From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [170]

    The Study Population section reports that none of the 84 patients with autoimmune hepatitis had antimitochondrial antibodies. The Clinical and Laboratory Assessments section describes antibody testing, but neither section addresses AMA in acute hepatitis or whether AMA titres usually decline over time. Searches for acute hepatitis and serial AMA titres found no passage supporting the claim. (LLM, conf=0.77)

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Research works printed but not linked: 1

  1. [11]11. Czaja AJ, Carpenter HA. Autoimmune hepatitis. In: MacSween RNM, Anthony PP, Scheuer PJ, Burt AD, Portmann B, Ishak KG, eds. Pathology of the Liver. 4th edition. Edinburgh: Churchill Livingstone (in press).

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Authors
Czaja, A. J., & Carpenter, H. A.
Affiliations
Mayo Clinic (US)
Corresponding author
Czaja, Albert J.
Venue
Hepatology
Published
2001-10
Volume
34
Issue
4
Pages
659-665
ISSN
0270-9139 (ISSN-L)
OpenAlex
W2095515459
Cited by
189
References
47
Citations by year
2025: 5 · 2024: 4 · 2023: 2 · 2022: 5 · 2021: 6 · 2020: 10 · 2019: 4 · 2018: 7 · 2017: 9 · 2016: 7 · 2015: 6 · 2014: 6 · 2013: 11 · 2012: 10
FWCI
5.71
Type
journal article
Language
en
Open access
Locations
  • Hepatology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
  • Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
  • Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Keywords
autoimmune hepatitis, bile duct injury, cholangitis, ductopenia, liver biopsy histology, treatment response, clinical outcomes, cholestatic syndrome
MeSH terms
Adolescent, Adult, Aged, Bile Ducts, Cholangitis, Female, Humans, Male, Middle Aged, Treatment Outcome, Hepatitis, Autoimmune

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