Digestive Diseases and Sciences

, Volume 56, Issue 1, pp 227–235

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


  • C. E. Eapen
    • Liver Unit, Queen Elizabeth Hospital
    • Department of GI SciencesLiver Unit, Christian Medical College
  • Peter Nightingale
    • Wellcome Trust Clinical Research FacilityQueen Elizabeth Hospital
  • Stefan G. Hubscher
    • Department of PathologyUniversity of Birmingham, Queen Elizabeth Hospital
  • Peter J. Lane
    • Clinical Immunology, Division of Immunology and InfectionUniversity of Birmingham, Queen Elizabeth Hospital
  • Timothy Plant
    • Department of ImmunologyUniversity of Birmingham, Queen Elizabeth Hospital
  • Dimitris Velissaris
    • Liver Unit, Queen Elizabeth Hospital
    • Department of Anaesthesiology and Intensive Care MedicineUniversity Hospital of Patras
    • Liver Unit, Queen Elizabeth Hospital
Original Article

DOI: 10.1007/s10620-010-1278-2

Cite this article as:
Eapen, C.E., Nightingale, P., Hubscher, S.G. et al. Dig Dis Sci (2011) 56: 227. doi:10.1007/s10620-010-1278-2



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.


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


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).


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.


Non-cirrhotic portal hypertensionCeliac diseaseInflammatory bowel diseaseIgA cardiolipin antibodyPortal vein thrombosis

Copyright information

© Springer Science+Business Media, LLC 2010