Laure Elkrief1,2,3, Marie Lazareth4, Sylvie Chevret5, Valérie Paradis3,6, Marta Magaz7, Lorraine Blaise8, Laura Rubbia-Brandt9, Lucile Moga4, François Durand4, Audrey Payancé4, Aurélie Plessier4, Cendrine Chaffaut5, Dominique Valla4, Marion Malphettes10, Alba Diaz11, Jean-Charles Nault8,12,13, Pierre Nahon8,12,13, Etienne Audureau14, Vlad Ratziu15, Laurent Castera4, Juan-Carlos Garcia Pagan7, Nathalie Ganne-Carrie8,12,13, Pierre-Emmanuel Rautou4. 1. Service de Transplantation et Hépato-gastroentérologie, Hôpitaux Universitaires de Genève, Switzerland. 2. Service d'Hépato-Gastroentérologie, CHU de Tours, France. 3. Université de Paris, Centre de recherche sur l'inflammation, Inserm, U1149, CNRS, ERL8252, Paris, France. 4. Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France. 5. Service de Biostatistiques et Information médicale, Hôpital Saint-Louis, APHP and Inserm, UMR-1153, ECSTRA Team, Paris, France. 6. Service d'Anatomopathologie, DHU Unity, DMU Digest, Hôpital Beaujon, AP-HP, Clichy, France. 7. Hepatic Hemodynamic Laboratory, European Reference Network for Rare Liver Disorders, Liver Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain. 8. AP-HP, Hôpital Jean Verdier, Liver Unit, Bondy, France. 9. Service d'anatomie et cytologie pathologiques, Hôpitaux Universitaires de Genève, Switzerland. 10. Service d'Immunopathologie clinique, Hôpital Saint-Louis, AP-HP, Paris, France. 11. Pathology Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain. 12. University Paris 13, Sorbonne Paris Cité, "équipe labellisée Ligue Contre le Cancer", Bobigny, France. 13. Inserm, UMR-1162 «Functional Genomics of Solid Tumors», Paris, France. 14. AP-HP, Hôpital Henri Mondor, Département de Santé Publique, and Université Paris-Est, A-TVB DHU, CEpiA (Clinical Epidemiology and Aging) Unit EA4393, UPEC, Créteil, France. 15. Service d'Hépato-gastroentérologie, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France.
Abstract
BACKGROUND AND AIMS: Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension. APPROACH AND RESULTS: Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD. CONCLUSIONS: This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.
BACKGROUND AND AIMS: Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension. APPROACH AND RESULTS: Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD. CONCLUSIONS: This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.