Pathogenesis and treatment of primary biliary cirrhosis

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Pathogenesis and
treatment of primary
biliary cirrhosis
Name: Eduard Miasnikov
Group: L1-226-2

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AIM: find information about primary biliary cirrhosis and provide
it in an accessible form.
Tasks:
1) Find keywords.
2) Generate relevant search questions to search for information.
3) Use search questions to search for articles.
4) Analysis of articles.
5) Formulation of conclusions.

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What is PBC?
Primary biliary cirrhosis (PBC) is an
idiopathic chronic autoimmune liver
disease that primarily affects
women. It is believed that the
aetiology for PBC is a combination
between environmental triggers in
genetically vulnerable persons.

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The diagnosis for PBC is made when two of the three criteria are
fulfilled and they are:
(1) biochemical evidence of cholestatic liver disease for at least 6 month's duration;
(2) anti-mitochondrial antibody (AMA) positivity;
(3) histologic features of PBC on liver biopsy;

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Incidence and prevalence
We identified 5,350 adults (15 years of age and older) with a PBC
diagnosis and 50,145 reference individuals. The PBC incidence rate
was around 2.6 per 100,000 person-years with some variability that
mainly can be explained by the start of different registers (a likely
overestimation 1987–1988, as well as 2001–2002), change in ICDcoding system and changes in care of patients. There was a steady
increase in the registered prevalence of PBC from 5.0 (1987) to 34.6
(2014) per 100,000 inhabitants, also with some variability due to the
difference in available registers. From 2004 to 2014 the data is
however likely to cover the full prevalent cohort of PBC diagnosed
patients, where the incidence is very stable, and the increase in
prevalence is close to linear.

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Incidence rates (per 100,000 person-years, red) and prevalence rates
(per 100,000 person-years, blue) for PBC in Sweden from 1987 to 2015.

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The median age of the whole study population was 64 years at
diagnosis (range 15–94), and 9 out of 10 patients were born in
Sweden. The female:male ratio in the whole study population was
4.0:1, with a higher proportion of women (5.4:1, 84.5%) in the
outpatient population 2001–2014.

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Data about authors, locations and publication dates of articles (NCBI)
1. Division of Gastroenterology and Hepatology, Mayo Clinic College
of Medicine, USA; 2010.
2. Collaborators: Inova Fairfax Hospital, Zucker School of Medicine at
Hofstra, Bon Secours Liver Institute of Virginia, Stanford University
Medical Center, Stanford University School of Medicine, Swedish
Medical Center, New York; 2019 Jan.

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Addresses of articles from the site of NCBI
https://pubmed.ncbi.nlm.nih.gov/20955967/
https://pubmed.ncbi.nlm.nih.gov/30429590/

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In fact, I can't find anything except a
description of what primary biliary cirrhosis is.
So there is no information on my topic here.

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Liver transplantation as a treatment method
The burden of cirrhosis is likely
underestimated, as patients with
compensated cirrhosis are frequently
asymptomatic and can remain
undiagnosed until decompensation.
Furthermore, liver transplantation (LT) is
the only curative treatment for
decompensated cirrhosis and acute liver
failure and serves as a treatment for
innate metabolic errors, liver cancer and
colorectal tumours with liver metastases.
The rise in patients with cirrhosis and
other clinical indications for LT is
concerning, as waiting lists grow.

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Untimely diagnosis
A timely diagnosis of cirrhosis is critical to initiate LT protocolization when appropriate.
However, referrals to a LT center is often delayed. Many patients with compensated
cirrhosis are asymptomatic, and therefore undiagnosed. Survey by Bittencourt and
colleagues found that ALD is incorrectly assumed to be the main cause of cirrhosis whereas
viral hepatitis was neglected, and a considerable proportion of surveyed citizens felt that
viral testing was unnecessary.

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Data about authors, locations and publication dates of articles (NCBI)
1.
Liver transplantation in Latin America: reality and challenges. David Aguirre-Villarreal,
Maximiliano Servin-Rojas, Aczel Sánchez-Cedillo, Mariana Chávez-Villa, Roberto HernandezAlejandro,Juan Pablo Arab, Isaac Ruiz, Karla P. Avendaño-Castro, Maria A. Matamoros,
Enrique Adames-Almengor, Javier Diaz-Ferrer,Erika Faride Rodriguez-Aguilar, Victor Manuel
Paez-Zayas, Alan G. Contreras, Mario R. Alvares-da-Silva, Manuel Mendizabal,Claudia P.
Oliveira," Miquel Navasa, and Ignacio García-Juárez. 2023;28

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Addresses of articles from the site of NCBI
https://www.thelancet.com/pdfs/journals/lanam/PIIS2667193X(23)00207-7.pdf

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Thanks of your attention