title: Pediatric ulcerative colitis associated with autoimmune diseases: a distinct form of inflammatory bowel disease?
abstract: BACKGROUND
  The pathogenesis of inflammatory bowel disease (IBD) is multifactorial, with some patients presenting additional autoimmune symptoms. Inflammatory colitis associated with autoimmune (AI) liver disease appears to have clinical features different from those of "classical" ulcerative colitis (CUC). The aim of this study was to describe these features, in order to differentiate a subgroup of colitis associated with autoimmunity (CAI) from CUC.
  
  
  METHODS
  Twenty-eight consecutive children with inflammatory colitis associated with primary sclerosing cholangitis (PSC), celiac disease, or AI hepatitis were compared with a matched control group of 27 children with isolated UC. Clinical course, histology, as well as inflammatory profile in the colonic mucosa based on real-time polymerase chain reaction (PCR) were analyzed.
  
  
  RESULTS
  In CAI the main digestive symptoms at disease onset were abdominal pain (12/28) and bloody strings in the stool (12/28), along with a high prevalence of autoimmune diseases in relatives, as compared with bloody diarrhea in the CUC group (26/27). At diagnosis, pancolitis was seen in 18/28 CAI patients compared with 8/27 in UC. In CAI, the pathological findings were different from CUC: 1) major lesions predominantly located in the right colon; 2) pseudo-villous appearance of the mucosa, and strong infiltration with eosinophils; 3) mild glandular lesions; and 4) differing inflammatory infiltrate with reduced FOXP3, interleukin (IL)-2, and thymic stromal lymphopoietin (TSLP) levels. Evolution in CAI was less aggressive, requiring less corticosteroids/immunomodulators.
  
  
  CONCLUSIONS
  Precise clinical, histological, and molecular analyses reveal marked differences between patients with CUC and those with associated AI phenomena, supporting the hypothesis of a distinct AI presentation of IBD.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Service de Gastroentérologie pédiatrique, Paris, France.
authors.forename: Felipe
authors.initials: F
authors.lastname: Ordonez
authors.forename: Florence
authors.initials: F
authors.lastname: Lacaille
authors.forename: Danielle
authors.initials: D
authors.lastname: Canioni
authors.forename: Cecile
authors.initials: C
authors.lastname: Talbotec
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Fournet
authors.forename: Nadine
authors.initials: N
authors.lastname: Cerf-Bensussan
authors.forename: Olivier
authors.initials: O
authors.lastname: Goulet
authors.forename: Jacques
authors.initials: J
authors.lastname: Schmitz
authors.forename: Frank M
authors.initials: FM
authors.lastname: Ruemmele
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000209:etiology* / Q000473:pathology; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003093:Colitis, Ulcerative / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies