Abstract

Background & 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 (TE-LSM) using transient elastography, 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 & results: Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to VALDIG criteria, to patients with compensated biopsy-proven cirrhosis related to alcohol (n=117), hepatitis C virus (HCV) infection (n=110) or non-alcoholic fatty liver disease (NAFLD) (n=46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, porto-systemic collaterals, history of ascites or platelet count < 150 x 109 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM [7.9 kilopascals (kPa)] than the patients with alcohol-, HCV- and NAFLD-related cirrhosis (33.8 kPa, 18.2 kPa, and 33.6 kPa, respectively; p<0.001). When compared to cirrhosis, a cut-off value of 10 kPa had a specificity of 97% for the diagnosis of PSVD, with a 85% positive predictive value. A cut-off value of 20 kPa had a sensitivity of 94% for ruling-out PSVD, with a 97% negative predictive value. 94% of the patients were well-classified. Even better results were obtained in a validation cohort including 78 PSVD patients.

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.

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