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Clinical Features and Risk Factors of Autoimmune Liver Involvement in Pediatric Inflammatory Bowel Disease
Matteo Bramuzzo1, Stefano Martelossi, Giuliano Torre
1*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.
Insights
Autoimmune liver disease, particularly sclerosing cholangitis, is more common in children with extensive ulcerative colitis. Monitoring is advised despite rare complications in pediatric patients.
Area of Science:
- Pediatric gastroenterology
- Hepatology
- Autoimmune diseases
Background:
- Autoimmune liver disease (AILD) affects up to 7.8% of pediatric patients with inflammatory bowel disease (IBD).
- A specific IBD phenotype associated with AILD has been observed in pediatric cohorts.
Purpose of the Study:
- To investigate the characteristics of IBD in children with co-occurring AILD.
- To analyze the specific features of the liver disease in these patients.
Main Methods:
- Data were sourced from the Italian Pediatric Inflammatory Bowel Disease Registry.
- Comparative analysis of patients with and without AILD was performed.
Main Results:
- AILD was present in 6.8% of 677 pediatric IBD patients.
- AILD was strongly linked to ulcerative colitis (83%), pancolonic involvement (84%), and perinuclear antineutrophil cytoplasmic antibody positivity (41%).
- Sclerosing cholangitis (61%) and overlap syndromes (33%) were common AILD definitions; 61% had intra- and extrahepatic biliary involvement.
Conclusions:
- AILD, especially sclerosing cholangitis, is significantly associated with extensive ulcerative colitis in children.
- Regular monitoring is recommended for pediatric patients with IBD and AILD due to potential hepatobiliary complications.
Objectives:
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.
Methods:
Information on patients was obtained from the Italian Pediatric Inflammatory Bowel Disease Registry. Data of patients with and without autoimmune liver disease were compared.
Results:
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.
Conclusions:
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.
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