Non-cirrhotic Intrahepatic Portal Hypertension: Associated Gut Diseases and Prognostic Factors

Overview

Journal Dig Dis Sci

Specialty Gastroenterology

Date 2010 May 26

PMID 20499175

Citations 33

Authors

C E Eapen

Peter Nightingale

Stefan G Hubscher

Peter J Lane

Timothy Plant

Dimitris Velissaris

Elwyn Elias

Affiliations

Soon will be listed here.

Abstract

Background/aims: Non-cirrhotic intrahepatic portal hypertension (NCIPH) is generally regarded to have a benign prognosis. We have studied a cohort followed-up at a tertiary referral center and postulate that gut-derived prothrombotic factors may contribute to the pathogenesis and prognosis of NCIPH.

Methods: We retrospectively analyzed prognostic indicators in 34 NCIPH patients. We also searched for associated gut diseases.

Results: Transplant-free survival in NCIPH patients from first presentation with NCIPH at 1, 5, and 10 years was 94% (SE: 4.2%), 84% (6.6%), and 69% (9.8%), respectively. Decompensated liver disease occurred in 53% of patients. Three (9%) patients had ulcerative colitis while five of 31 (16%) tested had celiac disease and on Kaplan-Meier analysis, celiac disease predicted reduced transplant-free survival (p=0.018). On multivariable Cox regression analysis, independent predictors of reduced transplant-free survival were older age at first presentation with NCIPH, hepatic encephalopathy, and portal vein thrombosis. Prevalence of elevated initial serum IgA anticardiolipin antibody (CLPA) was significantly higher in NCIPH (36% of patients tested), compared to Budd-Chiari syndrome (6%) (p=0.032, Fisher's exact test) and celiac disease without concomitant liver disease (0%) (p=0.007).

Conclusions: We have identified prognostic factors and report progression to liver failure in 53% of NCIPH patients followed-up at our center. Our data supports a role for intestinal disease in the pathogenesis of intrahepatic portal vein occlusion leading to NCIPH.

Citing Articles

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Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.

Shukla A, Rockey D, Kamath P, Kleiner D, Singh A, Vaidya A Hepatol Int. 2024; 18(6):1684-1711.

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A Narrative Review on Non-Cirrohotic Portal Hypertension: Not All Portal Hypertensions Mean Cirrhosis.

Fiordaliso M, Marincola G, Pala B, Muraro R, Mazzone M, Di Marcantonio M Diagnostics (Basel). 2023; 13(20).

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Idiopathic non-cirrhotic portal hypertension in a patient with Talaromyces marneffei infection: a case report.

Ye X, Quan X, Guo X, Wang Z, Wu H BMC Infect Dis. 2023; 23(1):125.

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