An international multisociety consensus statement has established a common definition and diagnostic framework for porto-sinusoidal vascular disorder (PSVD) and noncirrhotic portal fibrosis (NCPF), aiming to reduce longstanding variation in terminology and diagnosis across regions.
Published jointly in the Journal of Hepatology and several partner journals, the statement allows the terms PSVD and NCPF to be used interchangeably when the same diagnostic criteria are applied. It also introduces a scoring system that classifies cases as no diagnosis, possible PSVD/NCPF or definite PSVD/NCPF.
The framework addresses decades of inconsistent terminology and partially overlapping definitions. Portal hypertension without cirrhosis has been described using terms including idiopathic portal hypertension, obliterative portal venopathy and nodular regenerative hyperplasia, while diagnostic criteria have varied among major liver societies.
“We now have a clear, globally consistent and evidence-based definition of PSVD,” co-corresponding author Pierre-Emmanuel Rautou, MD, PhD, of the Department of Hepatology at Hôpital Beaujon, AP-HP, and Université Paris Cité, France, told GI & Hepatology News. “The score also provides granularity by distinguishing possible from definite PSVD.”
The initiative brought together adult and pediatric hepatologists and liver pathologists from societies across the Americas, Europe and Asia. Dedicated groups addressed biopsy adequacy, exclusion criteria, associated conditions and signs of portal hypertension, followed by structured voting and external Delphi validation.
The consensus defines PSVD/NCPF as a clinicopathological diagnosis requiring a high-quality liver biopsy, exclusion of cirrhosis and systematic assessment for specified alternative conditions before the score can be applied. The framework also recognizes that PSVD/NCPF may be diagnosed in the absence of clinically overt portal hypertension and may coexist with another liver disease or a recognized cause of cirrhosis, provided cirrhosis itself is not present on biopsy.
“The framework should facilitate diagnosis by making the criteria clearer and easier to apply,” Dr. Rautou said. “Everybody will speak the same language.”
The diagnostic score incorporates four domains: signs of portal hypertension, histological findings, conditions associated with PSVD/NCPF and concomitant causes of cirrhosis. Histological findings carry the greatest weight, followed by specific signs of portal hypertension. Scores of two or less indicate that PSVD/NCPF cannot be diagnosed on the available evidence. Scores of three or four indicate possible disease, while scores of five or more indicate definite PSVD/NCPF. Patients in the possible category may require further investigation, repeat biopsy, noninvasive assessment, follow-up or referral to a vascular liver disease center.
The score reproduced expert classifications across 36 hypothetical clinical scenarios and was endorsed by 87% of participants in an independent Delphi validation. “Liver biopsy is mandatory to calculate the score,” Dr. Rautou said. “Other tools, such as liver and spleen stiffness, are needed to raise suspicion. Once exclusion conditions have been ruled out and the biopsy is completed, the scoring system can be applied.”
The group defined an adequate biopsy for excluding cirrhosis as a specimen measuring at least 15 mm, with at least one fragment measuring 10 mm or longer. Major histological criteria include nodular regenerative hyperplasia, muscularized portal venules and portal venule stenosis involving at least half of portal tracts. The concurrent presence of all three minor histological criteria is considered equivalent to one major criterion.
The statement also standardizes signs of portal hypertension. Specific signs include variceal bleeding, medium or large esophageal varices, gastric varices and spontaneous portosystemic shunts. Ascites, small varices, thrombocytopenia and splenomegaly are classified as nonspecific signs. Noninvasive findings, including low liver stiffness combined with high spleen stiffness and certain magnetic resonance imaging features, may strengthen suspicion but do not add points to the score.
Implementation will depend heavily on consistent pathological assessment. The consensus replaces earlier references to “specific” histological lesions with standardized major and minor criteria, recognizing that individual abnormalities may also occur in other liver diseases. “A challenge now is to inform and train pathologists on the new histological criteria,” Dr. Rautou said.
The authors said the common terminology and scoring system should reduce diagnostic ambiguity, support more consistent clinical follow-up and improve comparisons across international research cohorts. Prospective evaluation will be needed to determine how the criteria perform in routine practice and outside specialist centers.
The work received public and academic research funding. Several authors reported consulting, advisory or speaking relationships with medical and pharmaceutical companies.