Original Article

Digestive Diseases and Sciences

, Volume 56, Issue 1, pp 227-235

First online:

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

  • C. E. EapenAffiliated withLiver Unit, Queen Elizabeth HospitalDepartment of GI Sciences, Liver Unit, Christian Medical College
  • , Peter NightingaleAffiliated withWellcome Trust Clinical Research Facility, Queen Elizabeth Hospital
  • , Stefan G. HubscherAffiliated withDepartment of Pathology, University of Birmingham, Queen Elizabeth Hospital
  • , Peter J. LaneAffiliated withClinical Immunology, Division of Immunology and Infection, University of Birmingham, Queen Elizabeth Hospital
  • , Timothy PlantAffiliated withDepartment of Immunology, University of Birmingham, Queen Elizabeth Hospital
  • , Dimitris VelissarisAffiliated withLiver Unit, Queen Elizabeth HospitalDepartment of Anaesthesiology and Intensive Care Medicine, University Hospital of Patras
  • , Elwyn EliasAffiliated withLiver Unit, Queen Elizabeth Hospital Email author 

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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.

Keywords

Non-cirrhotic portal hypertension Celiac disease Inflammatory bowel disease IgA cardiolipin antibody Portal vein thrombosis