A multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF

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dc.authorid0000-0001-7937-984X
dc.authorid0000-0003-3142-3762
dc.authorid0000-0002-4864-6498
dc.authorid0000-0001-5285-4460
dc.authorid0000-0003-1288-888X
dc.authorid0000-0003-2804-1165
dc.authorid0000-0002-2420-8573
dc.authorid0000-0002-8909-2102
dc.authorid0000-0001-9567-1859
dc.contributor.authorHernandez Gea, Virginia
dc.contributor.authorParadis, Valerie
dc.contributor.authorGuindi, Maha
dc.contributor.authorAlves, Venancio A.F.
dc.contributor.authorAqul, Amal
dc.contributor.authorCerda, Eira
dc.contributor.authorDarwish Murad, Sarwa
dc.contributor.authorDas, Prasenjit
dc.contributor.authorÖrmeci, Necati
dc.contributor.authorRautou, Pierre Emmanuel
dc.date.accessioned2026-05-04T17:12:23Z
dc.date.available2026-05-04T17:12:23Z
dc.date.issued2026
dc.departmentFakülteler, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, İç Hastalıkları Ana Bilim Dalı
dc.description.abstractNoncirrhotic portal hypertension has historically been described using heterogeneous and region-specific terminology—such as idiopathic portal hypertension (IPH), noncirrhotic portal fibrosis (NCPF), obliterative portal venopathy, and nodular regenerative hyperplasia—leading to substantial variability in diagnosis, reporting, and international research collaboration. Differences in guideline definitions from major societies (AASLD, EASL, and APASL), together with the presence of characteristic histologic lesions in patients without clinically overt portal hypertension, have further complicated disease classification. To address these challenges, a large, multisociety, international initiative was convened to harmonize nomenclature and diagnostic criteria. Representatives from liver, pathology, and pediatric hepatology societies across the Americas, Europe, and Asia participated in a structured consensus process that included specialized working groups and external Delphi validation. The initiative produced a globally harmonized and implementable diagnostic framework. Consensus was reached that the terms porto-sinusoidal vascular disorder (PSVD) and NCPF may be used interchangeably when identical diagnostic criteria are applied, and that they should be written as PSVD or NCPF. The diagnosis was defined as fundamentally clinicopathological, requiring integrated assessment. Core principles include the need for a high-quality liver biopsy ( ≥ 10 mm), mandatory exclusion of cirrhosis, and systematic exclusion of specific alternative conditions. Importantly, the consensus recognizes that PSVD or NCPF may be diagnosed even without clinical portal hypertension and may coexist with other liver diseases, provided cirrhosis is excluded. Standard ized major and minor histologic criteria were developed collaboratively by expert pathologists and externally validated. Features of portal hypertension were harmonized into specific and nonspecific categories applicable to routine clinical practice. An integrated diagnostic scoring system incorpo rating histology, clinical features, associated conditions, and concommitant etiologies was developed and validated using the Delphi method. This consensus provides the first internationally endorsed, unified framework for the diagnosis of PSVD or NCPF. Its global implementation is expected to reduce diagnostic variability, improve comparability across regions, and facilitate the development of robust, internationally harmonized clinical and translational research cohorts.
dc.identifier.citationHernandez Gea, V., Paradis, V., Guindi, M., Alves, V. A. F., Aqul, A., Cerda, E., Darwish Murad, S., Das, P., Örmeci, N., & Rautou, P. E. (2026). A multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF. Hepatology, pp. 1-16. https://doi.org/10.1097/HEP.0000000000001768
dc.identifier.doi10.1097/HEP.0000000000001768
dc.identifier.endpage16
dc.identifier.issn0270-9139
dc.identifier.issn1527-3350
dc.identifier.scopusqualityQ1
dc.identifier.startpage1
dc.identifier.urihttps://doi.org/10.1097/HEP.0000000000001768
dc.identifier.urihttps://hdl.handle.net/20.500.13055/1468
dc.identifier.wosqualityQ1
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.indekslendigikaynak.otherSCI-E - Science Citation Index Expanded
dc.institutionauthorÖrmeci, Necati
dc.institutionauthorid0000-0002-8909-2102
dc.language.isoen
dc.publisherAASLD
dc.relation.ispartofHepatology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectIdiopathic Portal Fibrosis
dc.subjectNodular Regenerative Hyperplasia
dc.subjectNoncirrhotic Portal Hypertension
dc.subjectPortal Hypertension
dc.subjectPortosinusoidal Vascular Disorder
dc.titleA multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF
dc.typeArticle
dspace.entity.typePublication

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