Idiopathic non-cirrhotic portal hypertension: a review
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REVIEW
Open Access
Idiopathic non-cirrhotic portal hypertension: a review Jeoffrey NL Schouten1, Joanne Verheij2 and Susana Seijo3*
Abstract Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. The etiology of INCPH can be classified in five categories: 1) immunological disorders (i.e. association with common variable immunodeficiency syndrome, connective tissue diseases, Crohn’s disease, etc.), 2) chronic infections, 3) exposure to medications or toxins (e.g. azathioprine, 6- thioguanine, arsenic), 4) genetic predisposition (i.e. familial aggregation and association with Adams-Oliver syndrome and Turner disease) and 5) prothrombotic conditions (e.g. inherited thrombophilias myeloproliferative neoplasm antiphospholipid syndrome). Roughly, INCPH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal hypertension. A formal diagnosis is based on the following criteria: 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases, and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging. Patients with INCPH usually present with signs or symptoms of portal hypertension such as gastro-esophageal varices, variceal bleeding or splenomegaly. Ascites and/or liver failure can occur in the context of precipitating factors. The development of portal vein thrombosis is common. Survival is manly limited by concomitant disorders. Currently, treatment of INCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied aimed to modify the natural history of the disease. Anticoagulation therapy can be considered in patients who develop portal vein thrombosis. Keywords: Non-cirrhotic portal hypertension, Portal hypertension, Variceal bleeding, Portal vein thrombosis
Introduction Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized by of intrahepatic portal hypertension in the absence of cirrhosis, other causes of liver disease and splanchnic venous thrombosis [1–7]. Histological features of INCPH comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal dilatation, phlebosclerosis and portal fibrosis to nodular regenerative hyperplasia. It is still unclear whether this repertoire of histological changes reflects different stages of the disease, or it could also be that INCPH comprises different nosologic entities that share the same clinical presentation. Different conditions have been associated to this disorder including immune-based diseases, recurrent infections, * Correspondence: [email protected] 3 Department of Medicine, CTO, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA Full list of author information is available at the end of
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