Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

Abstract

Idiopathic non-cirrhotic portal hypertension (INCPH) is a clinicopathologic disease entity characterized by the presence of clinical signs and symptoms of portal hypertension (PH) in the absence of liver cirrhosis or known risk factors accountable for PH. Multiple hematologic, immune-related, infectious, hereditary and metabolic risk factors have been associated with this disorder. Still, the exact etiopathogenesis is largely unknown. The recently proposed portosinusoidal vascular disease (PSVD) scheme broadens the spectrum of the disease by also including patients without clinical PH who are found to have similar histopathologic findings on core liver biopsies. Three histomorphologic lesions have been identified as specific for PSVD to include obliterative portal venopathy, nodular regenerative hyperplasia and incomplete septal cirrhosis/fibrosis. However, these findings are often subtle, under-recognized and subjective with low interobserver agreement among pathologists. Additionally, the natural history of the subclinical forms of the disease remains unexplored. The clinical course is more favorable compared to cirrhosis patients, especially in the absence of clinical PH or liver dysfunction. There are no universally accepted guidelines in regard to diagnosis and treatment of INCPH/PSVD. Hence, this review emphasizes the need to raise awareness of this entity by highlighting its complex pathophysiology and clinicopathologic associations. Lastly, formulation of standardized diagnostic criteria with clinical validation is necessary to avoid misclassifying vascular diseases of the liver and to develop and implement targeted therapeutic strategies.

Progressive dyspnoea in a patient with idiopathic non-cirrhotic portal hypertension

“…While the severity of PoPH may be graded by mPAP values (table 2), it is important to consider additional haemodynamic parameters and right heart function. Invasive measurement of hepatic venous pressure gradient (>5 mmHg) was considered the gold standard to diagnose portal hypertension, however, noninvasive techniques such as doppler ultrasonography are increasingly employed [14]. Furthermore, it should be noted that the hepatic venous pressure gradient may be normal in patients with extrahepatic portal hypertension.…”

Porto-Sinusoidal Vascular Disease in a Patient With Diffuse Aortitis and Massive Ascites

“…3 The 3 histologic findings identified in the spectrum of PSVD are OPV, nodular regenerative hyperplasia, and incomplete septal cirrhosis. 4…”

“…3 The 3 histologic findings identified in the spectrum of PSVD are OPV, nodular regenerative hyperplasia, and incomplete septal cirrhosis. 4 Patients with PSVD and INCPH may present with ascites, variceal bleeding, or encephalopathy, making it difficult to distinguish from cirrhosis without biopsy. Biochemical workup in PSVD typically reveals preserved liver function and aminotransferase levels 5 while demonstrating nonspecific abnormalities such as the presence of anti-nuclear antibodies.…”

Bioinformatics Analysis of Common Genetic and Molecular Traits and Association of Portal Hypertension with Pulmonary Hypertension

“…INCPH is a clinically pathological disease with the clinical features of portal hypertension after the exclusion of cirrhosis and known risk factors. However, with further study, portal sinus vascular disease was proposed, and the definition of INCPH was broadened to include the prephase of INCPH and the PH of known liver disease that are concomitant [ 9 , 10 ].…”