Guideline Update: PSVD-NCPF Nomenclature-Guideline Update: PSVD-NCPF Nomenclature

Video Transcription

Video Summary

Dr. Virginia Hernandez-Guea discusses an international effort to standardize the definition and diagnosis of porto-sinusoidal vascular disorder (PSVD), also known as non-cirrhotic portal fibrosis (NCPF). The two names remain in use as a compromise across regions, but the shared diagnostic criteria are the key advance, enabling researchers to combine patient cohorts and study the disease consistently.<br /><br />The revised criteria distinguish major and minor histological features, based on a systematic review by pathologists, and avoid labeling findings as “specific” when they can occur in other conditions. A diagnostic score also includes positive and negative points and a “possible” category for uncertain cases, who may need specialist assessment or follow-up.<br /><br />Hernandez-Guea emphasizes raising awareness, improving recognition among clinicians and pathologists, and defining endpoints for future trials. Priorities include merging global cohorts, investigating disease mechanisms and natural history, and studying pediatric cases.

Keywords

porto-sinusoidal vascular disorder

non-cirrhotic portal fibrosis

diagnostic criteria

histological features

clinical awareness

global cohort studies