Published online Sep 27, 2023. doi: 10.4254/wjh.v15.i9.1033
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
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.
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.
To evaluate the clinical profile, complications and survival rates of PSC patients submitted to LTx at a Brazilian referral center.
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.
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%.
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.
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.