Learning objectives
To expose the recent advances on understanding of cholangiopathies,
highlighting the pathophysiological aspects,
the new terms adopted and the etiologies currently involved with this pathology.
To discuss the subclassification of cholangiopathies,
explaining possible misunderstandings.
To illustrate the main imaging patterns of cholangiopathies that may be useful in narrowing the differential diagnosis.
Background
The recent advances in the understanding of cholangiopathies
The biliary tree is formed by intrahepatic and extrahepatic bile ducts,
is lined by biliary epithelial cells (the cholangiocytes) and also has peribiliary glands around the intrahepatic large bile ducts and extrahepatic bile ducts.
Cholangiopathies are considered to be all pathologies affecting the biliary tree and targeting cholangiocytes.
In general,
cholangiopathies lead to a change in normal cholangiocyte function,
and encompass a series of diseases that are commonly chronic,
many without effective treatment,
progressive and potentially fatal....
Findings and procedure details
Now that we have reviewed the key concepts about cholangiopathies,
we can exemplify them dividing into: genetic,
immune-mediated,
idiopathic,
infectious,
vascular and obstructive.
Genetic
Caroli Disease
Definition: Caroli disease,
or communicating ectasia of the intrahepatic bile ducts,
is a rare and autosomal recessive disorder that is part of the clinicopathologic spectrum of ductal plate malformations.
If the large intrahepatic bile ducts are affected,
the result is Caroli disease,
whereas abnormal development of the small intrahepatic bile ducts results in congenital hepatic fibrosis.
If all levels...
Conclusion
Despite the overlapping features,
imaging findings are fundamental in diagnosing cholangiopathies.
Since some etiologies are potentially reversible and even curable if correctly managed,
the proper diagnosis is essential.
Personal information
Contact details:
Hanna R.
F.
Dalla Pria
Department of Imaging,
Universidade Federal de São Paulo
Rua Napoleão de Barros,
800 - Vila Clementino
CEP 04024-002 - São Paulo/SP,
Brazil.
Email:
[email protected]
Author Information
Hanna R.
F.
Dalla Pria (Department of Imaging,
Universidade Federal de São Paulo,
São Paulo,
Brazil).
Fernanda Velloni (Department of Imaging,
Universidade Federal de São Paulo,
São Paulo,
Brazil;Diagnósticos da América S.A.
DASA.
São Paulo,
SP,
Brazil).
Rafael A.
Santiago(Department of Imaging,
Universidade Federal de São Paulo,
São Paulo,
Brazil;Diagnósticos da América...
References
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Pathobiology of biliary epithelia.
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2017 Jul;35(5):1256–68.
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Levy,
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Diseases of the Gallbladder and Bile Ducts.
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