Minireviews
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World J Hepatol. Apr 27, 2023; 15(4): 538-553
Published online Apr 27, 2023. doi: 10.4254/wjh.v15.i4.538
Morphological aspects of small-duct cholangiopathies: A minireview
Eva Sticova, Ondrej Fabian
Eva Sticova, Ondrej Fabian, Clinical and Transplant Pathology Centre, Institute for Clinical and Experimental Medicine, Prague 14021, Czech Republic
Eva Sticova, Department of Pathology, The Third Faculty of Medicine, Charles University and University Hospital Kralovske Vinohrady, Prague 10000, Czech Republic
Ondrej Fabian, Department of Pathology and Molecular Medicine, The Third faculty of Medicine, Charles University and Thomayer University Hospital, Prague 14059, Czech Republic
Author contributions: Sticova E wrote the manuscript and prepared the figures and tables; Fabian O provided input on the writing of the paper and prepared the figures; Both authors revised and edited the draft and are in agreement with the content of the manuscript.
Conflict-of-interest statement: All the authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Eva Sticova, MD, PhD, Associate Professor, Doctor, Clinical and Transplant Pathology Centre, Institute for Clinical and Experimental Medicine, IKEM, Videnska 1958/9, Prague - 4, Prague 14021, Czech Republic. eva.sticova@ikem.cz
Received: December 28, 2022
Peer-review started: December 28, 2022
First decision: January 22, 2023
Revised: February 3, 2023
Accepted: March 22, 2023
Article in press: March 22, 2023
Published online: April 27, 2023
Processing time: 112 Days and 23.9 Hours
Abstract

The biliary system consists of intrahepatic and extrahepatic bile ducts lined by biliary epithelial cells (cholangiocytes). Bile ducts and cholangiocytes are affected by a variety of disorders called cholangiopathies, which differ in aetiology, pathogenesis, and morphology. Classification of cholangiopathies is complex and reflects pathogenic mechanisms (immune-mediated, genetic, drug- and toxin-induced, ischaemic, infectious, neoplastic), predominant morphological patterns of biliary injury (suppurative and non-suppurative cholangitis, cholangiopathy), and specific segments of the biliary tree affected by the disease process. While the involvement of large extrahepatic and intrahepatic bile ducts is typically visualised using radiology imaging, histopathological examination of liver tissue obtained by percutaneous liver biopsy still plays an important role in the diagnosis of cholangiopathies affecting the small intrahepatic bile ducts. To increase the diagnostic yield of a liver biopsy and determine the optimal therapeutic approach, the referring clinician is tasked with interpreting the results of histopathological examination. This requires knowledge and understanding of basic morphological patterns of hepatobiliary injury and an ability to correlate microscopic findings with results obtained by imaging and laboratory methods. This minireview describes the morphological aspects of small-duct cholangiopathies pertaining to the diagnostic process.

Keywords: Bile duct; Cholangiocytes; Cholangiopathy; Cholangitis; Liver biopsy; Histopathology

Core Tip: A wide range of non-neoplastic and neoplastic conditions affects the small intrahepatic bile ducts. Cholangiopathies account for significant morbidity and mortality and represent an important indication for liver transplantation in adult and paediatric populations. Pathogenesis of most cholangiopathies is complex, and likely involves both environmental and genetic factors. Understanding the underlying pathogenetic mechanisms, knowledge of basic morphological patterns, and an ability to correlate microscopic findings with results obtained by imaging and laboratory methods are important steps in forming an overall clinical picture and selecting the optimal therapeutic approach in patients with biliary diseases. Our minireview addresses some important morphological aspects of small-duct cholangiopathies relevant to the diagnostic process, and also provides a brief overview of the most clinically significant conditions in light of recent progress.