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Disease severity prognostication in primary sclerosing cholangitis

Disease severity prognostication in primary sclerosing cholangitis

Study Overview:

  • Title: Disease severity prognostication in primary sclerosing cholangitis: a validation of Anali scores and comparison with the potential functional stricture
  • Objective: To propose MRI-based Anali scores (with and without contrast) and potential functional stricture (PFS) scores as a measure for predicting disease progression in PSC patients.
  • Type of Study: Retrospective, single-center study.
  • Study Population: The study analyzed MRI scans from 123 adults (average age 40.5 years) with confirmed large-duct PSC. Individuals with small duct PSC, a history of liver transplant, unrelated diseases (e.g., lung cancer, secondary sclerosing cholangitis, etc.), or those under the age of 18 were excluded from this study.
  • Research Institution/Team: Led by clinicians at the Medical University of Vienna, collaborating with clinicians and researchers from the Mayo Clinic, Karolinska Institute, Hannover Medical School, Assistance Publique - Hopitaux de Paris, and Sorbonne University.

Key Findings

  • Gap in knowledge: 
    • There are few non-invasive prognostic values with perfect agreement among clinicians for PSC diagnosis and progression. Development of a prognostic tool on routine imaging for PSC patients provides a method to address this gap while reducing burden on patient quality of life.
  • Background:
    • Anali score was developed and validated as a risk scoring system for MRI to predict progression of PSC. This scoring system was introduced in 2014 (PMID: 23857427) and validated in 2019 (PMID: 30880273). Poor to moderate agreement between radiologists in validation study, emphasizing importance of validating amongst multi-center cohorts to assess clinical impact of this scoring.
    • Potential functional stricture (PFS) is the name for either physical narrowing of bile ducts due to scar tissue development or due to hepatocellular dysfunction, both of which are characteristic of PSC. This nomenclature helps clinicians find ways to describe observations found through imaging, like MRI, to increase prognostic value. This term was initially proposed in PSC by this study in 2023 (PMID: 37470827)
  • Key Results:
    • The combined PFS and ANALI score on contrast-enhanced MRI can stratify PSC patients into low-risk and high-risk categories for disease complications.
    • The ANALI score on non-contrast MRI in PSC patients is a strong predictor of adverse outcomes, such as liver failure, the need for a transplant, or death.
    • The identification of a PFS indicates an increased likelihood of adverse events in PSC patients.

Data and Evidence:

  • Among the scoring methods, ANALINoGd showed the strongest prediction of adverse event-free survival in low-risk versus high-risk PSC patients (HR 6.12). PFS (HR 3.12) and contrast-enhanced Anali scores (ANALIGdAP: HR 3.79, ANALIGdHBP: HR 3.66) also indicated strong predictive ability, as an HR of 1 shows no difference in adverse event probabilities between risk groups.
  • Non-contrast ANALI scoring method strongly predicted event-free survival of PSC patients through classification of low-risk and high-risk groups.
  • PFS had the highest inter-reader agreement (0.81) and intra-reader agreement (0.87, 0.82), indicating it is a reliable measure among radiologists.
  • Among the scoring methods validated, PFS had the most consistent results among radiologist, emphasizing its reliability on detecting strictures in PSC patients. 
  • The non-contrast Anali score (ANALINoGd) had a 95.2% negative predictive value (likelihood of experiencing adverse events).

Relevance to the PSC Community

  • Impact on PSC Community: Validated findings empower clinicians with a non-invasive, MRI-based tool to more effectively diagnose diseases and predict their severity and outcomes. This supports earlier, informed decisions about treatment options or transplant referrals, enhancing the quality of care.
  • Practical Applications: This work will educate PSC patients about their diagnosis, enabling them to make informed decisions about their care and monitoring. Low-risk patients may avoid unnecessary invasive procedures, while early intervention may be considered for detected PFS to prevent liver damage.
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