Portal sinusoidal vascular diseases: Assessment and therapy

Abstract

The term porto-sinusoidal vascular disease (PSVD) was introduced in 2019 to describe a group of liver conditions that can lead to portal hypertension (PH) in the absence of cirrhosis or portal vein thrombosis, with or without specific findings on liver histology. The new nomenclature has facilitated the consolidation of knowledge on diseases previously referred to by various terms, including Banti’s disease, non-cirrhotic portal hypertension, non-cirrhotic portal fibrosis, and idiopathic portal hypertension, while excluding certain etiologies like sarcoidosis, congenital hepatic fibrosis, and Budd-Chiari syndrome. The prevalence and recognition of the disorder has been increasing. Advances in diagnostics and treatment have improved life expectancy for patients with associated conditions, such as immunodeficiencies and autoimmune diseases. Similar to cirrhosis, patients with PSVD may experience complications of PH, including variceal bleeding and ascites. However, less is known about its natural history, screening strategies, prognosis, and treatment options. This review discusses methods for assessing PSVD, including clinical and histological features, imaging techniques, and currently available treatments. It also addresses the challenges posed by the new nomenclature and the remaining questions in disease assessment.