Idiopathic non-cirrhotic portal hypertension

The pathogenesis of idiopathic non-cirrhotic portal hypertension(INCPH) remains unknown and the disease is diagnosed by the absence of recognized clinical indicators of cirrhosis and of any other known etiologies of portal hypertension.To promote understanding of this disease,a comprehensive overview of potential etiologies,clinical manifestations,histopathological features,methods of diagnosis and potential differential diagnoses,and outcome of clinical management is presented in this review.In particular,we discuss the findings from INCPH studies and their implications in regards to each of the above-mentioned categories.For example,associations with various comorbidities have suggested a possible immune system component to INCPH development and/or progression.In addition,the common clinical characteristics of patients upon presentation can not only help to recognize disease suspects but may also provide insights into the pathogenesis and prognosis.Finally,prognosis following the various intervention strategies appears to depend mainly on severity of the portal hypertension,as well as its various accompanying complications.