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2015 ; 10
(ä): 67
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Idiopathic non-cirrhotic portal hypertension: a review
#MMPMID26025214
Schouten JN
; Verheij J
; Seijo S
Orphanet J Rare Dis
2015[May]; 10
(ä): 67
PMID26025214
show ga
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.
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