Background and aims: Hepatoportal sclerosis (HPS) is a clinicopathologic condition that is clinically characterized by portal hypertension (varices and portosystemic collateral vessels), splenomegaly and pancytopenia, in the absence of cirrhosis. Although the etiology is obscure, a number of theories such as immunologic and vascular endothelial cellular abnormalities have been put forward to explain the underlying pathophysiology. Angiotensin-converting enzyme (ACE), an important molecule of the renin-angiotensin system (RAS), is also known as a regulatory molecule in systemic and portal circulation in distinct disorders. The aim of the present study was to investigate the possible role of the ACE in the context of RAS in HPS pathogenesis.
Materials and methods: The study was conducted on 30 HPS patients (16 men, 14 women; median age 36 years, range 18-63) and 20 healthy controls. The clinical features of HPS patients including demographics, laboratory, and ultrasonography findings were summarized. Serum ACE levels were measured by using commercially available kits.
Results: Serum median ACE levels were 36 (8-174) U/l and 16 (8-43) U/l for the HPS patients and controls, respectively. Serum ACE levels were significantly higher in patients with HPS compared to the control group (P < 0.05).
Conclusion: ACE in the context of RAS may be associated with pathological endothelial occlusive events in the microenvironment of the portal circulation in HPS. Revealing the interactions between circulating and local RAS within the hepatic microenvironment would enlighten the biologic basis and clinical management of liver diseases.