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
Abstract
BACKGROUND

Primary sclerosing cholangitis (PSC) manifests within a broad ethnic and racial spectrum, reflecting different levels of access to health care.

AIM

To evaluate the clinical profile, complications and survival rates of patients with PSC undergoing liver transplantation (LTx) at a Brazilian reference center.

METHODS

All patients diagnosed with PSC before or after LTx were included. The medical records were reviewed for demographic and clinical variables, including outcomes and survival. The level of statistical significance was set at P < 0.05.

RESULTS

Our cohort represented 1.6% (n = 34) of the 2113 patients receiving liver grafts at our service over the past two decades. Most were male (n = 19; 56%). The average age (40 ± 14 years) was similar for men and women (P = 0.347). The mean follow-up time from diagnosis to LTx was 68 mo. Most patients had the classic form of PSC. Three women had PSC/autoimmune hepatitis overlap syndrome, and one patient had small-duct PSC. Alkaline phosphatase levels at diagnosis and pre-LTx model for end-stage liver disease. scores were significantly higher in males. Inflammatory bowel research (IBD) was investigated by colonoscopy in 26/34 (76%) and was present in most cases (18/26; 69%). IBD was less common in women than in men (44.4% vs. 55.6%) (P = 0.692). Cholangiocarcinoma (CCA) was diagnosed in 2/34 (5.9%) patients by histopathology of the explant (survival: 3 years 6 mo, and 4 years 11 mo). Two patients had complications requiring a second LTx (one after 7 d due to hepatic artery thrombosis and one after 17 d due to primary graft dysfunction). Five patients (14.7%) developed biliary stricture. The overall median post-LTx survival was 66 mo. Most deaths occurred in the first year (infection n = 2, primary liver graft dysfunction n = 3, unknown cause n = 1). The 1-year and 5-year survival rates of this cohort were 82.3% and 70.6%, respectively, matching the mean overall survival rates of LTx patients at our center (87.1% and 69.43%, respectively) (P = 0.83).

CONCLUSION

Survival after 1 and 5 years was similar to that of other LTx indications. The observed CCA survival rate suggests CCA may be an indication for LTx in selected cases.

Keywords: Primary sclerosing cholangitis, Epidemiology, Liver transplantation, Survivor, Clinical associations, Pathological features

Core Tip: We present a case series of liver transplantation (LTx) patients from the largest center in Northeastern Brazil, with epidemiological features different from what is expected for primary sclerosing cholangitis (PSC) (e.g., early manifestation and proportion of female patients). The finding of two cases of cholangiocarcinoma (CCA) with good survival is relevant to the discussion on the eligibility of selected cases of CCA for LTx. The survival of PSC patients was similar to that of LTx patients with other etiologies.