new project: 2D-SWE to detect PSVD

[Pierre-Emmanuel Rautou]

Dear VALDIG friends,
We are happy to propose a new collaborative project entitled “Liver and spleen stiffness by 2D-shear-wave elastography to detect porto-sinusoidal vascular liver disorder in patients with signs of portal hypertension”.
You can find below the project.
If you are interested, please send us an email and we will send you the CRF and the project in PDF (for the moment, we can’t add the project to VALDIG website for technical reasons).
Best wishes

Lucile Moga, lucile.moga@gmail.com
Pierre-Emmanuel Rautou, pierre-emmanuel.rautou@inserm.fr

Background
In 2019, VALDIG proposed the term of porto-sinusoidal vascular liver disease (PSVD) to describe a group of rare vascular liver entities characterized by histological lesions involving portal venules and/or sinusoids and absence of cirrhosis, with or without signs of portal hypertension (1). Recently, the Baveno VII consensus workshop preferred the term “disorder” instead of “disease” so that PSVD now stands for “porto-sinusoidal vascular liver disorder”. PSVD is defined as follows:
• Adequate liver biopsy (≥20 mm long, and not too fragmented, or considered adequate for interpretation by an expert pathologist) or liver explant without cirrhosis AND one sign specific for portal hypertension or one histological lesion specific for PSVD;
• Or adequate liver biopsy (≥20 mm long, and not too fragmented, or considered adequate for interpretation by an expert pathologist) or liver explant without cirrhosis AND one sign not specific for portal hypertension AND one histological lesion not specific for PSVD.

Histology is therefore mandatory for the diagnosis of PSVD, with a high quality liver biopsy, and an interpretation by an expert pathologist. Non-invasive tests appear useful tools to screen for PSVD. Elkrief et al. showed that liver stiffness measurement using transient elastography is able to raise suspicion of PSVD in patients with signs of portal hypertension with high accuracy (2). Ferreira-Silva et al. found that spleen stiffness measurement using transient elastography has a good performance in predicting high-risk varices in patients with non-cirrhotic portal hypertension (3), but this result was not observed by other groups (4).

Aims
• To evaluate feasibility and accuracy of two-dimensional liver and spleen shear-wave elastography (2D-SWE) to discriminate porto-sinusoidal vascular liver disorder (PSVD) from cirrhosis in patients with signs of portal hypertension.
• To compare accuracy of 2D-SWE to that of transient elastography – liver stiffness measurement (TE-LSM) to detect PSVD with signs of portal hypertension.

Design
Part I: Learning group of patients with PSVD
The learning cohort will include the 153 patients with PSVD who underwent a liver biopsy between 2012 to 2021 at Hôpital Beaujon (Clichy, France) and a liver 2D-SWE. All liver biopsies were analyzed by a pathologist expert in vascular liver diseases (Prof. Valérie Paradis, Hôpital Beaujon, Clichy, France). Diagnosis of PSVD will be based on VALDIG criteria, as stated in Baveno VII consensus.

Part II: Validation group of patients with PSVD
The validation cohort will include PSVD patients from participating VALDIG centers fulfilling the same inclusion criteria.

Part III: First control group of patients with cirrhosis
Patients with cirrhosis and signs of portal hypertension will be selected at each VALDIG centers participating in the study, matched 1:1 on ascites (absent; moderate or controlled with diuretics; tense or requiring paracentesis), with patients with PSVD.

Part IV: Second control group of patients with cirrhosis
For liver 2D-SWE: patients with cirrhosis included in the international multicentre cohort study on 2D-SWE in cirrhosis by Trebicka et al. (Gut . 2022 Feb;71(2):402-414), matched 1:1 on ascites (absent; moderate or controlled with diuretics; tense or requiring paracentesis), with patients with PSVD (from learning and validation cohorts). These patients with cirrhosis should not be part of the first control group. Investigators who provided their data for the paper mentioned above (Gut . 2022 Feb;71(2):402-414) will be contacted to ask for permission to used their data in the present study.
For spleen 2D-SWE: patients with cirrhosis included in the ongoing meta-analysis of individual participant data to determine the most performant cut-offs of spleen stiffness measurement (SSM) for the prediction of portal hypertension and/or esophageal varices in patients with compensated advanced chronic disease coordinated by Prof. Antonio Colecchia. Patients will be matched 1:1 on ascites (absent; moderate or controlled with diuretics; tense or requiring paracentesis), with patients with PSVD (from learning and validation cohorts). These patients with cirrhosis should not be part of the first control group. Investigators who provided their data for the paper mentioned above (meta-analysis of individual participant data coordinated by Prof. Antonio Colecchia) will be contacted to ask for permission to used their data in the present study.

 
References
1. De Gottardi A, Rautou P-E, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity. Lancet Gastroenterol Hepatol. mai 2019;4(5):399‑411.
2. Elkrief L, Lazareth M, Chevret S, Paradis V, Magaz M, Blaise L, et al. Liver Stiffness by Transient Elastography to Detect Porto-Sinusoidal Vascular Liver Disease With Portal Hypertension. Hepatology. juill 2021;74(1):364‑78.
3. Ferreira-Silva J, Gaspar R, Liberal R, Cardoso H, Macedo G. Transient splenic elastography predicts high-risk esophageal varices in patients with non-cirrhotic portal hypertension. Scand J Gastroenterol. déc 2021;56(12):1462‑6.
4. Cunningham ME, Parastandeh-Chehr G, Cerocchi O, Wong DK, Patel K. Noninvasive Predictors of High-Risk Varices in Patients with Non-Cirrhotic Portal Hypertension. Can J Gastroenterol Hepatol. 2019;2019:1808797.
5. Trebicka J, Gu W, de Ledinghen V, Aubé C, Krag A, Praktiknjo M, et al. Two-dimensional shear wave elastography predicts survival in advanced chronic liver disease. Gut. févr 2022;71(2):402‑14.

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