Title: Disease severity prognostication in primary sclerosing cholangitis: a validation of Anali scores and comparison with the potential functional stricture
Background:
- Few non-invasive prognostic tools for PSC have demonstrated strong inter-clinician agreement in diagnosis or predicting disease progression. Developing imaging-based scoring systems offers a promising way to address this gap while also reducing burden on patients’ quality of life.
- The ANALI score was introduced as an MRI-based risk scoring system to predict PSC progression and later underwent validation. However, its validation showed poor to moderate agreement between radiologists, emphasizing the need for further assessment in multi-center cohorts to evaluate its clinical utility and reliability.
- The term Potential Functional Stricture (PFS) refers to either physical narrowing of bile ducts due to scar tissue development or due to hepatocellular dysfunction, both of which are key characteristics of PSC.
- This concept offers clinicians a standardized framework for interpreting MRI findings, thereby improving prognostic accuracy.
Objective: The goal of the study was to evaluate the utility of MRI-based ANALI scores—both with and without contrast—as well as Potential Functional Stricture (PFS) scoring, as metrics for predicting disease progression in patients with PSC.
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 (Italy), Mayo Clinic (United States of America), Karolinska Institute (Sweden), Hannover Medical School (Germany), Assistance Publique - Hopitaux de Paris (France), and Sorbonne University (France).
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.
- Non-contrast ANALI scoring method strongly predicted event-free survival of PSC patients through classification of low-risk and high-risk groups.
- Among the scoring methods validated, PFS had the most consistent results among radiologists, emphasizing its reliability on detecting strictures in PSC patients.
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.
- 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.
