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.
Highlights
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 [1,2,3,4,5,6,7]
Criteria and differential diagnosis The diagnosis of INCPH is a diagnosis of exclusion, based on the following previously reported criteria [1]: 1) presence of unequivocal signs of portal hypertension; 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases that can cause PH by appropriate serological, biochemical tests and liver biopsy and; 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging studies performed at diagnosis
Some genetic traits have been identified in Human immunodeficiency virus (HIV)-associated INCPH [25]
Summary
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 [1,2,3,4,5,6,7]. A recent multicenter study demonstrated a genetic predisposition to develop INCPH in HIV infected patients chronically exposed to didanosine [4, 15, 25]. Despite these data, it is difficult to assign a definitive etiopathogenic role of didanosine, as the drug has been widely used for the treatment of HIV in the past. It is tempting to blame drug intake and chemical exposure as primary etiological factors, only a very small proportion of the patients treated with the above mentioned drugs or exposed to these chemicals develop clinical or histological signs of INCPH. Additional factors may play a pathogenic role in these patients
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