Liver Stiffness by Transient Elastography to Detect Porto‐Sinusoidal Vascular Liver Disease With Portal Hypertension

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 × 10 9 /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.