Objectives: Idiopathic portal hypertension (IPH) is the presence of PH in the absence of liver disease. Recently, IPH was reported in HIV patients on highly active antiretroviral therapy (HAART). We describe for the first time the hepatic and systemic hemodynamic profile, liver stiffness, and histological features of eight patients with HIV-related IPH.
Methods: HIV-positive patients were identified from our IPH database over 3 years.
Results: Five patients presented with variceal bleeding, two with splenomegaly, and one with ascites. All had large esophageal varices. Median hepatic venous pressure gradient (HVPG) was 8 mm Hg (range 3.5-14.5), clearly underestimating the true portal pressure. This is probably because of a presinusoidal component of PH and because of the presence of intrahepatic venous collaterals. Median liver stiffness was 8.9 kPa (range 6.8-14.9) and was unreliable in predicting the presence of fibrosis or of esophageal varices. The main histological features were absence of portal vein radicles and areas of regenerating hepatocytes. Six patients (75%) developed portal vein thrombosis during a 2-year follow-up.
Conclusions: There is a subset of HIV patients without cirrhosis but with PH compatible with IPH. In these patients, the hepatic and systemic hemodynamic profile is similar to other forms of IPH. The histological profile reflects an underlying vascular disorder affecting the medium-sized portal vein branches. Development of portal vein thrombosis is a frequent complication and requires close monitoring.