Porto-sinusoidal vascular disease——A novel entity of portal hypertension

Porto-sinusoidal vascular disease (PSVD) is an entity characterized with portal hypertension (PH) in the absence of cirrhosis, the related risk factors, and imaging evidence of obstructed portal vein, hepatic vein and inferior vena cava. Its prevalence varies significantly between East and West countries. Until now, the etiologies have been classified as autoimmune, hematologic, and prethrombotic conditions, infections, toxins or drugs, and genetic or metabolic disorders. However, the definite cause remains unknown. Diagnosis is based on three histopathological features: obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The clinical manifestations of early PSVD are nonspecific, whereas those at a late stage are similar to cirrhosis. The imaging detection mainly reveals the PH signs and complications, but the liver stiffness is normal or slightly increased, necessitating a liver biopsy for PVSD diagnosis. PSVD treatment is similar to liver cirrhosis; however, the prognosis is better. In order to gain a thorough understanding of PSVD, the epidemiology, pathogenesis, clinical diagnosis, and treatment are discussed in this article.