A NEW international consensus on noncirrhotic portal fibrosis (NCPF) diagnosis could help standardise the identification of a poorly understood cause of noncirrhotic portal hypertension, addressing longstanding inconsistencies that have complicated clinical practice and research collaboration across regions.
NCPF, also known as porto-sinusoidal vascular disorder (PSVD) is a vascular liver disease that can lead to portal hypertension in the absence of cirrhosis, although some patients may not have clinically obvious portal hypertension. The condition has historically been described using multiple terms, including idiopathic portal hypertension, obliterative portal venopathy and nodular regenerative hyperplasia, creating substantial variation in diagnosis and reporting.
The newly developed framework brought together international experts to establish harmonised nomenclature and diagnostic criteria. The group agreed that PSVD and NCPF are interchangeable terms when the same diagnostic criteria are used and should be referred to as PSVD or NCPF.
Unified PSVD/NCPF Diagnosis Framework Introduced
The consensus defines PSVD diagnosis as a clinicopathological process requiring integrated assessment rather than a single clinical or histological feature.
They concluded that it was necessary to exclude cirrhosis and specific alternative conditions. Experts agreed unanimously that a high-quality biopsy measuring 15 mm, with at least one fragment of 10 mm or greater, is sufficient to reliably exclude cirrhosis when PSVD or NCPF is suspected.
Importantly, the framework recognises that PSVD or NCPF may be diagnosed even in the absence of clinically overt portal hypertension. The disorder can also coexist with other liver diseases, provided cirrhosis has been excluded.
Scoring System Seeks to Reduce Diagnostic Variability
To support more consistent classification, investigators developed and externally validated a diagnostic scoring system. Clinical evidence of portal hypertension contributes between 0 and 3 points depending on severity, while histological findings contribute up to 5 points. Conditions previously reported in association with PSVD or NCPF add 1 point, whereas conditions known to cause cirrhosis to reduce the score by 1 point.
Scores of 3–4 indicate possible PSVD or NCPF and may warrant follow-up or referral to an expert centre. A score of 5 or higher confirms the diagnosis, while scores of 2 or below are considered insufficient to support a diagnosis.
Addressing Longstanding Research Challenges
Differences between guidance issued by major liver societies have previously hindered efforts to compare international cohorts and advance understanding of disease mechanisms.
Despite decades of recognition, PSVD remains poorly characterised at the biological and mechanistic levels. Research has largely consisted of observational studies, reflecting marked clinical and histological heterogeneity. Although a shared pathogenic process has been proposed to link disorders grouped under the PSVD umbrella, definitive evidence remains lacking.
Global adoption of the new framework could improve diagnostic consistency, facilitate internationally harmonised research cohorts and support future translational studies aimed at clarifying disease mechanisms and informing therapeutic strategies.
Reference
Hernandez-Gea et al. A multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF. Ann Hepatol. 2026;DOI:10.1016/j.aohep.2026.102219.
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