Porto-sinusoidal Vascular Disease: Classification and Clinical Relevance

Abstract

Non-cirrhotic portal hypertension (NCPH) is a well-recognized clinico-pathological entity, which is associated with clinical signs and symptoms, imaging, and endoscopic features of portal hypertension (PHT), in absence of cirrhosis. In patients with NCPH without known risk factors of PHT or extrahepatic portal vein thrombosis, the condition is called idiopathic non-cirrhotic portal hypertension (INCPH). There are multiple infectious, immune related causes, systemic diseases, drug and toxin exposures, haematological disorders, and metabolic risk factors that have been associated with this INCPH. However, the causal pathogenesis is still unclear. The Vascular liver disorders interest group group recently proposed porto-sinusoidal vascular disease (PSVD) as a syndromic entity, which provides definite histopathological criteria for diagnosis of NCPH (table 1). The three classical histo-morphological lesions specific for PSVD include obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The PSVD definition includes patients with portal vein thrombosis, PVT, and even those without PHT, thus broadening the scope of diagnosis to include patients who may have presented early, prior to haemodynamic changes consistent with PHT. However, this new diagnosis has pros and cons. The cons include mandating invasive liver biopsy to assess the PSVD histological triad in all patients with NCPH, an erstwhile clinical diagnosis in Asian patients. In addition, the natural history of the subclinical forms of PSVD without PHT and linear progression to develop PHT is unknown yet. In this review, we discuss the diagnosis and treatment of INCPH/PSVD, fallacies and strengths of the old and new schema, pathobiology of this disease, and clinical correlates in an Asian context. Although formulation of standardised diagnostic criteria is useful for comparison of clinical cohorts with INCPH/PSVD, prospective clinical validation in global cohorts is necessary to avoid misclassification of vascular disorders of the liver.

Keywords: NCPH; PSVD; extrahepatic portal vein obstruction; non-cirrhotic portal hypertension; portal hypertension; portosinusoidal vascular disease.

Figure 1

Conditions clearly associated with NCPH causing changes at pre-sinusoidal, sinusoidal, and post-sinusoidal levels. In patients in whom there are no competing causative factors, and liver biopsy in consistent, a diagnosis of idiopathic NCPH or INCPH is made. Abbreviations: INCPH = idiopathic non-cirrhotic portal hypertension; NCPH = non-cirrhotic portal hypertension.

Figure 2

Diagnostic algorithm for porto-sinusoidal vascular disease.

Figure 3

Systemic disorders, infections, metabolic, immunological factors, and drugs or toxins associated with PSVD. Abbreviation: PSVD = porto-sinusoidal vascular disease.

Figure 4

Overlapping conditions in the INCPH and PSVD cohorts to show disorders common to both and exclusions in the definitions. Abbreviations: INCPH = idiopathic non-cirrhotic portal hypertension; PSVD = porto-sinusoidal vascular disease.