title: Non-cirrhotic intrahepatic portal hypertension: associated gut diseases and prognostic factors.
abstract: OBJECTIVE
  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.
authors.affiliation: Liver Unit, Queen Elizabeth Hospital, and Department of Pathology, University of Birmingham, Edgbaston, Birmingham, B15 2TH, UK. eapen@cmcvellore.ac.in
authors.forename: C E
authors.initials: CE
authors.lastname: Eapen
authors.forename: Peter
authors.initials: P
authors.lastname: Nightingale
authors.forename: Stefan G
authors.initials: SG
authors.lastname: Hubscher
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Lane
authors.forename: Timothy
authors.initials: T
authors.lastname: Plant
authors.forename: Dimitris
authors.initials: D
authors.lastname: Velissaris
authors.forename: Elwyn
authors.initials: E
authors.lastname: Elias
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017153:Antibodies, Anticardiolipin / Q000097:blood; D001706:Biopsy; D002446:Celiac Disease / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D015331:Cohort Studies; D003093:Colitis, Ulcerative / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000473:pathology; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012044:Regression Analysis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult
chemical_list: D017153:Antibodies, Anticardiolipin; D016682:Lupus Coagulation Inhibitor