Premium
Histological features in western patients with idiopathic non‐cirrhotic portal hypertension
Author(s) -
Verheij Joanne,
Schouten Jeoffrey N L,
Komuta Mina,
Nevens Frederik,
Hansen Bettina E,
Janssen Harry L A,
Roskams Tania
Publication year - 2013
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12114
Subject(s) - portal hypertension , medicine , cirrhosis , pathological , fibrosis , portal vein thrombosis , portography , pathology , gastroenterology , radiology
Aims In the western world, idiopathic non‐cirrhotic portal hypertension ( INCPH ) is a rare disease. This study aimed to investigate the histopathological features in western INCPH patients and to assess pathological differences between liver specimens of INCPH with and without HIV . Methods and results Biopsies of 70 INCPH patients (of which 15 were HIV ‐infected) were compared to 23 patients with non‐cirrhotic portal vein thrombosis ( PVT ), which served as a control group for non‐cirrhotic portal hypertension. Phlebosclerosis, nodular regeneration ( NR ), sinusoidal dilatation, paraportal shunting vessels, perisinusoidal fibrosis and portal tract remnants were the most prevalent morphological features of INCPH . There were significant ( P < 0.01) morphological differences between INCPH and PVT liver specimens with regard to portal tract remnants (46% versus 0%), phlebosclerosis (95% versus 65%), portal vein dilatation (34% versus 78%) and NR (56% versus 22%). The degree of NR correlated with the severity of phlebosclerosis ( P < 0.01). NR was seen more frequently in the HIV – INCPH group, compared to the non‐ HIV ‐infected patients ( P < 0.001). Conclusion Portal tract remnants, phlebosclerosis and nodular regeneration are typical features of INCPH . Sinusoidal dilatation, paraportal shunting vessels and increased portal and parenchymal vessels might represent pressure‐related morphological signs of portal hypertension. Finally, more nodular regeneration was observed in HIV ‐associated INCPH .