Journal article
Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome
Annals of diagnostic pathology, Vol.67, 152178
12/2023
DOI: 10.1016/j.anndiagpath.2023.152178
PMID: 37468373
Abstract
The diagnosis of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and AIH-PBC overlap syndrome (OS) relies on their histologic features and clinical findings. In this study, we aimed to identify specific morphologic features of these diseases and evaluate their clinical correlation.
We included initial biopsies from untreated patients with AIH (n = 14), PBC (n = 10), and OS (n = 7). Histologic features of the portal tract, portal-lobular interface, and hepatic lobule, fibrosis, as well as clinical data including serology, autoantibodies, treatment, and prognosis were reviewed and analyzed.
Our results showed that several histologic features differed significantly between AIH and PBC (p < 0.05). Among these features, OS cases were more likely to present with bile duct-centered processes (presence of bile duct damage while absence of inflammation gradient from bile duct to interface, plasma cell cluster and pericentral inflammation) unlike those seen in AIH (p < 0.05), and interface-centered processes (unequivocal interface hepatitis, ductular reaction, and periportal fibrosis) which were not seen in PBC (p < 0.05). We observed a significant correlation between transaminase levels and lobular inflammation, including numbers of lymphocyte, plasma cell and eosinophil. Our study also found that anti-smooth muscle antibody positivity was associated with interface hepatitis (p < 0.01), while antimitochondrial antibody positivity was associated with duct damage (including ductopenia) and granulomas (p < 0.05).
Our results highlight distinctive morphological features between AIH and PBC. The possibility of overlap syndrome should be considered when encountering AIH with bile duct-centered processes or PBC with interface-centered processes in morphology and correlation with autoantibodies.
•There are distinctive morphological features among autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and AIH-PBC overlap syndrome (OS).•The histologic features of AIH, PBC and OS are correlated with transaminase levels and status of autoantibodies.•OS cases are more likely to present both bile duct-centered and interface-centered processes.
Details
- Title: Subtitle
- Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome
- Creators
- Yuanxin Liang - Yale UniversityBinny Khandakar - Yale UniversityYansheng Hao - University of Rochester Medical CenterYiqin Xiong - University of IowaBella L. Liu - Icahn School of Medicine at Mount SinaiXuchen Zhang - Yale University
- Resource Type
- Journal article
- Publication Details
- Annals of diagnostic pathology, Vol.67, 152178
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.anndiagpath.2023.152178
- PMID
- 37468373
- ISSN
- 1092-9134
- eISSN
- 1532-8198
- Language
- English
- Electronic publication date
- 07/12/2023
- Date published
- 12/2023
- Academic Unit
- Pathology
- Record Identifier
- 9984445524202771
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