Idiopathic non-cirrhotic portal hypertension (INCPH)
- Idiopathic non-cirrhotic portal hypertension (INCPH) is a vascular liver disease of unknown etiology.
- Clinical signs of portal hypertension (PHT) (esophageal varices, low platelet count, and splenomegaly) develop in the absence of cirrhosis with preserved liver function.
- By definition, occlusion of the extrahepatic portal vein (PV)/hepatic veins, any disorder known to induce PHT in the absence of cirrhosis (e.g., infiltrative diseases, vascular malignancies, schistosomiasis, congenital hepatic fibrosis, sarcoidosis), and any known cause of chronic liver disease (CLD) must be excluded to make a diagnosis of INCPH.
- Association with drugs and toxins (azathioprine, 6-thioguanine, vinyl chloride and arsenic); infections (HIV and chronic infections of the gut); immunological disorders (congenital immunodeficeincy disorders and autoimmune diseases), genetic etiology (Familial obliterative portal venopathy, Adams-Oliver syndrome and Turner’s syndrome) and miscellaneous (chronic hemodialysis and cystic fibrosis-related liver disease).
- Various terms have been used to describe this disorder: non-cirrhotic portal fibrosis (NCPF) idiopathic portal hypertension (IPF), nodular regenerative hyperplasia (NRH), incomplete septal cirrhosis, partial nodular transformation, obliterative portal venopathy, and hepatoportal sclerosis.
- Liver biopsy: Constellation of features in various combinations such as non-cirrhotic nodular transformation, obliterative portal venopathy, phlebosclerosis, herniated portal veins (ectatic veins), hisshapened portal veins, loss of portal veins or increased number of portal veins, pseudo-angiomatosis, paraportal shunts, megasinusoids, parenchymal atrophy – portal tracts approximating each other, parenchymal hyperplasia – portal tracts distant from each other, increased number of draining central veins and incomplete septal cirrhosis.
- Prognosis: Very few studies have evaluated the long-term prognosis of INCPH patients. Overall, prognosis appears to be generally better than that of patients with cirrhosis.
References
- Schouten JNL, Verheij J, Seijo S. Idiopathic non-cirrhotic portal hypertension: a review. Orphanet J Rare Dis. 2015; 10:67.
- Schouten JNL, Garcia‐Pagan JC, Valla DC, Janssen HLA. Idiopathic noncirrhotic portal hypertension. Hepatology. 2011;54:1071-81.
NextGenPath Archives
