Factors Associated with Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System
For patients with primary sclerosing cholangitis (PSC), and the clinicians treating them, it can be very difficult to predict the future. The course of PSC varies greatly between different individuals, with some patients at much lower risk of requiring future liver transplantation than others. Quantifying this risk has, until recently, been difficult, due to the absence of large enough studies in this area.
Recently published research (December 2018) by Dr Elizabeth Goode of the University of Cambridge and UK-PSC, examined more than 1,000 patients with PSC, recruited to the UK-PSC cohort, in the biggest detailed study of risk factors for outcomes in PSC, to date. Dr Goode and her team identified clinical factors available from diagnosis that can predict those patients with PSC who will survive without the need for transplantation (known as transplant-free survival) and reports a risk scoring system that can be used to quantify these risks.
The scoring system, designed to be used by clinicians and researchers, is split into two parts. Firstly, the short-term risk score (RSST), calculated at the time of diagnosis, predicts transplant-free survival two-years following diagnosis and secondly, the long-term risk score (RSLT) calculated at 2 years following diagnosis, which predicts the likelihood of transplant-free survival at 10-years following diagnosis.
What factors are associated with longer transplant-free survival in PSC?
- Lower blood values of bilirubin and alkaline phosphatase (ALP)
- Higher values of albumin, haemoglobin and platelets count
- The presence of disease confined to the intra-hepatic bile ducts, and not affecting the extra-hepatic bile ducts
- The absence of bleeding from oesophageal varices in the first 2 years following diagnosis
Why is this research so important?
This scoring system can provide reassurance to many patients and their clinicians, that their disease course is likely to be mild. For those patients with a more severe disease type, at higher risk of requiring liver transplantation, it can help guide entry into clinical trials of new treatments, or early referral on to specialist treatment centres with expertise in managing severe PSC.
Read more about how PSC doctors predict what will happen to you.
This study is published in the journal ‘Hepatology’.
The risk score calculator is available for use by clinicians and researchers at the UK-PSC website. Please note, the UK-PSC Risk Scores Tool is provided as an information resource only and is not to be used or relied upon for any diagnostic or treatment purposes.