Table of contents
Idiopathic noncirrhotic portal hypertension
Background
Overview
Definition
INCPH is a rare liver disorder characterized by an increased portal venous pressure gradient in the absence of a known cause of liver disease and portal vein thrombosis.
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Pathophysiology
The pathophysiology of INCPH is complex and not fully understood. However, three main mechanisms have been proposed: increased resistance to blood flow in the portal vein, abnormal angiogenesis leading to the formation of portosystemic collaterals, and sinusoidal endothelial dysfunction.
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Epidemiology
INCPH is a rare disease in the Western world, with a higher prevalence in India.
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Disease course
Clinical manifestations of INCPH can include recurrent ascites, splenomegaly, variceal bleeding, and portal vein thrombosis. It can also lead to serious complications such as liver failure, HCC, and portal hypertensive gastropathy.
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Prognosis and risk of recurrence
The prognosis of INCPH is generally favorable as it does not typically lead to liver failure.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of idiopathic noncirrhotic portal hypertension are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016).
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