Porto-sinusoidal vascular disorder, report of a novel association with POEMS syndrome. Future challenge for the hepatologist

Abstract

Porto-Sinusoidal Vascular Disorder (PSVD) is a recently introduced clinical entity. Since it is rare and often underrecognized, there is growing interest in identifying patients at increased risk. We present a case of a 59-years-old male with refractory ascites, pleural effusion, and high-risk varices meeting the diagnostic criteria for PSVD with a concomitant diagnosis of POEMS syndrome. The possible association between PSVD and POEMS syndrome has been described only in eight reports in literature, but it may be underrecognized due to the clinical manifestations overlap. To gain a wider comprehension of PSVD, it is fundamental to cooperate using international networks.

Keywords: Idiopathic non cirrhotic portal hypertension; POEMS syndrome; PSVD; Porto-sinusoidal vascular disorder.