A multisociety consensus statement on a new common definition and diagnostic criteria for PSVD or NCPF
Year: 2026
Type: article
Abstract: Non-cirrhotic portal hypertension has historically been described using heterogeneous and region-specific terminology, such as idiopathic portal hypertension (IPH), non-cirrhotic 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. Standardized 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 incorporating 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.
Source: Journal of Hepatology
Authors: Virginia Hernández-Gea, Valerie Paradis, Maha Guindi, Venancio A.F. Alves, Amal Aqul, Eira Cerda, Sarwa Darwish Murad, Prasenjit Das, Angelo Di Giorgio, Luiz A.R. de Freitas, Tassos Grammatikopoulos, Kenichi Harada, Nelia Hernandez, Samar H. Ibrahim, Sanjay Kakar, Saul Karpen, David E. Kleiner, Necati Ormeci, Xiaolong Qi, Puja Sakhuja, Maria Isabel Schinoni, Romil Saxena, Alexandre Sayadi, Akash Shukla, Dina G. Tiniakos, Elizabeth Verna, Kerry Wong, Laure Elkrief, Christine Sempoux, Theo Heller, Pierre-Emmanuel Rautou
Institutions: Hospital Clínic de Barcelona, Consorci Institut D'Investigacions Biomediques August Pi I Sunyer, Fundació de Recerca Clínic Barcelona-Institut d’Investigacions Biomèdiques August Pi i Sunyer, Inserm, Université Paris Cité, Hôpital Beaujon, Assistance Publique – Hôpitaux de Paris, Centre de Recherche sur l'Inflammation, Cedars-Sinai Medical Center, Universidade de São Paulo, Hospital Alemão Oswaldo Cruz, The University of Texas Southwestern Medical Center, Hospital Central Militar, Erasmus MC, All India Institute of Medical Sciences, University of Udine, Ospedale Santa Maria della Misericordia di Udine, Universidade Federal da Bahia, Fundação Oswaldo Cruz, King's College London, King's College Hospital, Kanazawa University, Hospital de Clínicas, Mayo Clinic, University of California, San Francisco, Virginia Commonwealth University, National Cancer Institute, Center for Cancer Research, Istanbul Health and Technology University, Zhongda Hospital Southeast University, Fundació Clínic per a la Recerca Biomèdica, Southeast University, Complexo Hospitalar Universitário Professor Edgard Santos, Emory University, Sir H.N. Reliance Foundation Hospital and Research Centre, National and Kapodistrian University of Athens, Aretaeio Hospital, Newcastle University, Columbia University, University of Alberta, Centre Hospitalier Universitaire de Tours, Centre Hospitalier Universitaire Vaudois, University of Lausanne, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases
Language: English
Subfield: Hepatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $5,500
Funders: U.S. Department of Health and Human Services, Emory University, University of Texas Southwestern Medical Center, Virginia Commonwealth University, Kanazawa University, University of Alberta, King's College London, Newcastle University, European Commission, National and Kapodistrian University of Athens, Universidade de São Paulo, Universidad de la República Uruguay, Université de Lausanne, Centre Hospitalier Universitaire Vaudois, Fundação Oswaldo Cruz, Southeast University, Università degli Studi di Udine, Fakultet Medicinskih Nauka, Univerziteta U Kragujevcu, National Institutes of Health, School of Medicine, Emory University, University of California, San Francisco, Instituto de Salud Carlos III, Faculty of Medical Sciences, Newcastle University, National Cancer Institute, National Institute of Diabetes and Digestive and Kidney Diseases
OpenAlex ID: W7162415150