Retrospective Cohort Study
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Sep 27, 2023; 15(9): 1033-1042
Published online Sep 27, 2023. doi: 10.4254/wjh.v15.i9.1033
Liver transplant in patients with primary sclerosing cholangitis: A retrospective cohort from Northeastern Brazil
Louyse Teixeira de Souza Freitas, Elodie Bomfim Hyppolito, Victor Leonardo Barreto, Luiz Humberto Jataí Castelo Júnior, Bianca Carneiro de Melo Jorge, Frederico César Tahim de Sousa Brasil Háteras, Malthus Barbosa Marzola, Clébia Azevedo Lima, Raquel Mendes Celedonio, Gustavo Rêgo Coelho, Jose Huygens Parente Garcia
Louyse Teixeira de Souza Freitas, Elodie Bomfim Hyppolito, Clébia Azevedo Lima, Raquel Mendes Celedonio, Gustavo Rêgo Coelho, Jose Huygens Parente Garcia, Liver Transplant Department, Walter Cantídio Teaching Hospital, Fortaleza 60430270, Ceará, Brazil
Elodie Bomfim Hyppolito, Victor Leonardo Barreto, Luiz Humberto Jataí Castelo Júnior, Bianca Carneiro de Melo Jorge, Frederico César Tahim de Sousa Brasil Háteras, Malthus Barbosa Marzola, School of Medicine, University of Fortaleza, Fortaleza 60811905, Ceará, Brazil
Elodie Bomfim Hyppolito, Hospital São José, Ceará State Health Department, Fortaleza 60455610, Ceará, Brazil
Gustavo Rêgo Coelho, Jose Huygens Parente Garcia, Department of Surgery, Federal University of Ceará, Fortaleza 60430140, Ceará, Brazil
Gustavo Rêgo Coelho, Jose Huygens Parente Garcia, Surgery Department, São Carlos Hospital, Fortaleza 60130241, Ceará, Brazil
Author contributions: Freitas LTS and Hyppolito EB designed the study; Freitas LTS, Hyppolito EB, Barreto VL, Júnior LHJC, Jorge BCM, Háteras FCTSB and Marzola MB reviewed the literature, collected data and drafted the original manuscript; Hyppolito EB performed statistical analyses and reviewed the manuscript; Hyppolito EB, Coelho GR, Garcia JHP, Lima CA and Celedonio RM reviewed the intellectual content of the manuscript; all authors read and approved the final version of the manuscript.
Institutional review board statement: The study was reviewed and approved by the ethics committee of the Federal University of Ceará and filed under Approval No. 98627218.6.2018.5045.
Conflict-of-interest statement: The authors declare they have no conflicts of interest.
Data sharing statement: The dataset available from the corresponding author at louyseteixeira.s@gmail.com.
STROBE statement: The authors declare they have read the STROBE statement. The present manuscript was prepared and revised following the checklist of the STROBE statement.
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: Louyse Teixeira de Souza Freitas, BSc, Nurse, Liver Transplant Department, Walter Cantídio Teaching Hospital, 1142 Coronel Nunes de Melo Street, Fortaleza 60430270, Ceará, Brazil. louyseteixeira.s@gmail.com
Received: May 7, 2023
Peer-review started: May 7, 2023
First decision: June 7, 2023
Revised: July 7, 2023
Accepted: August 25, 2023
Article in press: August 25, 2023
Published online: September 27, 2023
ARTICLE HIGHLIGHTS
Research background

Primary sclerosing cholangitis (PSC) is a rare indication for liver transplantation (LTx). Male sex is predominant in European studies. The ideal moment for LTx can be difficult to determine. PSC is often associated with inflammatory bowel disease (IBD) and may recur after LTx.

Research motivation

A Brazilian multicenter study on PSC showed that LTx patient data are limited and little explored in research. Our LTx service is the largest in North/Northeastern Brazil, with an average of 150 procedures a year, indicating a potential for research. The diagnosis of IBD in PSC patients before and after LTx is often inadequate and requires more attention on part of LTx teams. The finding of associated cholangiocarcinoma (CCA) in explants, associated with good survival, was an additional motivating factor.

Research objectives

To evaluate the clinical profile, complications and survival rates of PSC patients submitted to LTx at a Brazilian referral center.

Research methods

Retrospective study of medical records supplemented by telephone interviews with patients. The study contributed to setting up a database of PSC patients submitted to LTx at our service.

Research results

PSC was observed in 1.6% of LTx patients. Male sex was predominant, but the proportion of women was considerably higher than in the literature. Women were diagnosed later than men, but PSC was more severe in men, including CCA in explants. The prevalence of IBD was 73%. PSC was diagnosed later in IBD patients. The median time from the diagnosis of IBD to the diagnosis of PSC was 9.8 years. Diabetes was significantly more common in patients without IBD. Aspartate transferase was 1.6 times higher in PSC patients with IBD. Esophageal varices were more frequent in non-IBD patients. The most prevalent treatment before LTx was ursodeoxycholic acid. Most men (88%) were treated endoscopically for dominant stenosis prior to LTx. CCA was an incidental finding in two patients with satisfactory survival. The survival of our PSC patients was better than that of LTx patients with other indications at our service. Survival was 81.9% (1 year) and 78.8% (5 years). PSC recurred in 5.88%.

Research conclusions

In our cohort of 34 PSC patients submitted to LTx (2002-2023), the proportion of women was unusually high. CCA patients had satisfactory survival, despite the recurrence of PSC. In patients with both PSC and IBD, the disease was less severe.

Research perspectives

Our study raises the hypothesis that early-stage CCA may be an acceptable indication for LTx. The observed differences in severity in the male sex and the high proportion of women in the cohort require further investigations into the genetic profile of this population.