Letter: non‐cirrhotic portal hypertension and end‐stage liver disease – authors’ reply

Abstract

We thank Dr Sharma for his comments on our paper.1, 2 Indeed we and others demonstrate that a subset of patients with idiopathic noncirrhotic portal hypertension (INCPH) develop liver failure.3, 4 However, in our series at INCPH diagnosis, no significant pathological differences between patients developing liver failure (n = 12) and those with remaining normal liver function (n = 50) could be identified. Furthermore, only 2 of 4 INCPH patients with liver failure outside the setting of intercurrent conditions were transplanted. Histopathological examination of these 2 liver specimens demonstrated phlebosclerosis, nodular transformation, sinusoidal dilatation and hypoplastic portal tracts. Severe fibrosis or cirrhosis was not observed. Larger multicentre studies will be needed to examine the association between histopathological features and the development of liver failure in INCPH patients, mainly as the association between benign nodular regeneration and a more severe spectrum of the disease has been reported inconsistently.4 Additionally, in transplant resection specimens, a heterogeneity of lesions has been reported.3 Unfortunately, due to small numbers, we could not identify clinical differences between patients developing hepatic encephalopathy after transjugular intrahepatic portosystemic shunt placement and persons undergoing surgical portosystemic shunting. The authors’ declarations of personal and financial interests are unchanged from those in the original article.2