title: Clinical Features and Risk Factors of Autoimmune Liver Involvement in Pediatric Inflammatory Bowel Disease.
abstract: Autoimmune liver disease is reported in up to 7.8% of children with inflammatory bowel disease. A distinct inflammatory bowel disease phenotype has been suggested in adults and in small pediatric cohorts. The aim of the study was to evaluate the features of inflammatory bowel disease associated with autoimmune liver diseases and to analyze the characteristics of the liver disease.
  
  
  
  Information on patients was obtained from the Italian Pediatric Inflammatory Bowel Disease Registry. Data of patients with and without autoimmune liver disease were compared.
  
  
  
  Autoimmune liver disease was detected in 6.8% of the 677 patients enrolled and was significantly associated with the diagnosis of ulcerative colitis (83%), with pancolonic involvement (84%), and with perinuclear antineutrophil cytoplasmic antibody positivity (41%) (all Ps < 0.05). Autoimmune liver disease was defined as sclerosing cholangitis in 61% of the patients and as an overlap syndrome in 33%. Concomitant intra- and extrahepatic biliary involvement was reported in 61% of cases, whereas exclusive extrahepatic lesions were reported in 21%. Hepatobiliary complications were observed in 9% of the patients during follow-up.
  
  
  
  Autoimmune liver disease, especially sclerosing cholangitis, was significantly more common in patients with extensive ulcerative colitis. Although complications are relatively rare in the pediatric age, monitoring is recommended.
authors.affiliation: *Paediatric Department, Gastroenterology and Nutrition Unit, Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste †Hepatology, Gastroenterology and Nutrition Unit, Bambino Gesù Children's Hospital, Rome ‡Paediatric Gastroenterology Unit, Institute "Giannina Gaslini", Genoa §Paediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Rome ||Paediatric Department, Children's Hospital "V. Buzzi," Milan ¶Unit of Paediatric Gastroenterology and Digestive Endoscopy, Department of Paediatrics Hospital "S.Spirito", Pescara #Paediatric Gastroenterology Unit, Department of Women and Children's Health, Padua University Hospital, Padua **Paediatric Gastroenterology and Cystic Fibrosis Unit, University of Messina, Messina ††Paediatric Gastroenterology and Endoscopy, Regional Center for IBD, Pediatric Department, University of Messina, Messina ‡‡Paediatric Gastroenterology and Hepatology, University of Pisa, Pisa §§Clinical Epidemiology and Public Health Research Unit, Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.
authors.forename: Matteo
authors.initials: M
authors.lastname: Bramuzzo
authors.forename: Stefano
authors.initials: S
authors.lastname: Martelossi
authors.forename: Giuliano
authors.initials: G
authors.lastname: Torre
authors.forename: Sabrina
authors.initials: S
authors.lastname: Cardile
authors.forename: Serena
authors.initials: S
authors.lastname: Arrigo
authors.forename: Silvia
authors.initials: S
authors.lastname: Vignola
authors.forename: Federica
authors.initials: F
authors.lastname: Ferrari
authors.forename: Giovanna
authors.initials: G
authors.lastname: Zuin
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Illiceto
authors.forename: Marco
authors.initials: M
authors.lastname: Gasparetto
authors.forename: Salvatore
authors.initials: S
authors.lastname: Pellegrino
authors.forename: Claudio
authors.initials: C
authors.lastname: Romano
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Maggiore
authors.forename: Marcella
authors.initials: M
authors.lastname: Montico
authors.forename: Marina
authors.initials: M
authors.lastname: Aloi
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000209:etiology*; D003093:Colitis, Ulcerative / Q000150:complications*; D003424:Crohn Disease / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D012042:Registries; D012307:Risk Factors